Education/Credentials Research Fellowship National : Jewish Center and Medicine University of Colorado Health Sciences Center (Medicine, Pulmonary and Critical Care ) Clinical Fellowship : University of Colorado Health Sciences Center (Pulmonary and Critical Care Medicine )Chief Residency : University of Michigan (Internal Medicine )Internship and Residency : University of Michigan (Internal Medicine )Medical Degree: University of Texas Medical Branch (Medicine)Bachelor's Degree (magna cum laude): University of California, San Diego (Chemistry) Board Certifications & LicensesAmerican Board of Internal Medicine (Certification Date: 1985-09-11)American Board of Internal Medicine (Pulmonary Disease) (Certification Date: 1988-11-01) SpecialtiesCritical Care MedicineInternal Medicine Contact Information Medical Sciences Building 6262 Office 513-558-4831 Email mccormfx@ucmail.uc.edu Research InterestsBasic laboratory focus 1. The structure/function relationships of surfactant proteins, surfactant homeostasis, surfactant proteins (collectins) in host defense. 2. Mechanisms of pulmonary fibrosis 3. The role of the pulmonary epithelium in influenza infection 4. Molecular and cellular basis of pulmonary alveolar microlithiasis 5. Pathogenesis of pulmonary Langerhans histiocytosis Clinical research focus 1. Genetic interstitial lung disea... Clinical Interests Critical Care MedicineLung DiseasePulmonary DiseaseRare Lung DiseaseSarcoidosisAsbestosisBronchiolitisChronic CoughDyspneaHistoplasmosisIdiopathic Pulmonary FibrosisLegionnaires' Disease (Legionellosis)Lymphangioleiomyomatosis (LAM)Lymphatic DiseasePleurisyPneumoniaPneumonitisPulmonary FibrosisPulmonary SarcoidosisRespiration DisordersRespiratory Syncytial Virus (RSV)Respiratory Tract DiseaseRespiratory Tract InfectionsShortness of BreathThoracic DiseaseTuberculosisWhooping Cough (Pertussis) Peer Reviewed Publications Gibbons, Erin; Taya, Manisha; Wu, Huixing; Lopa, Samia H; Moss, Joel; Henske, Elizabeth P; McCormack, Francis X; Hammes, Stephen R 2024. Glycoprotein non-metastatic melanoma protein B promotes tumor growth and is a biomarker for lymphangioleiomyomatosis. Endocrine-related cancer, 31 6, Gomez-Arroyo, Jose; Houweling, Arjan C; Bogaard, Harm Jan; Aman, Jurjan; Kitzmiller, Joseph A; Porollo, Aleksey; Dooijes, Dennis; Meijboom, Lilian J; Hale, Phillip; Pauciulo, Michael W; Hong, Jason; Zhu, Na; Welch, Carrie; Shen, Yufeng; Zacharias, William J; McCormack, Francis X; Aldred, Micheala A; Weirauch, Matthew T; Graf, Stefan; Rhodes, Christopher; Chung, Wendy K; Whitsett, Jeffrey A; Martin, Lisa J; Kalinichenko, Vladimir V; Nichols, William C 2024. Role of Forkhead box F1 in the Pathobiology of Pulmonary Arterial Hypertension. bioRxiv : the preprint server for biology, , Hasegawa, Yoshihiro; Franks, Jennifer M; Tanaka, Yusuke; Uehara, Yasuaki; Read, David F; Williams, Claire; Srivatsan, Sanjay; Pitstick, Lori B; Nikolaidis, Nikolaos M; Shaver, Ciara M; Kropski, Jonathan; Ware, Lorraine B; Taylor, Chase J; Banovich, Nicholas E; Wu, Huixing; Gardner, Jason C; Osterburg, Andrew R; Yu, Jane J; Kopras, Elizabeth J; Teitelbaum, Steven L; Wikenheiser-Brokamp, Kathryn A; Trapnell, Cole; McCormack, Francis X 2024. Pulmonary osteoclast-like cells in silica induced pulmonary fibrosis. Science advances, 10 28, eadl4913Jose, Arun; Rahman, Natalia; Opotowsky, Alexander R; Glorioso, Thomas J; Waldo, Stephen W; Zeder, Katarina; Seto, Arnold; Elwing, Jean M; McCormack, Francis X; Maron, Bradley A 2024. Association of Cardiopulmonary Hemodynamics and Mortality in Veterans With Liver Cirrhosis: A Retrospective Cohort Study. Journal of the American Heart Association, 13 8, e033847Rubin, Rhea; Baldi, Bruno Guedes; Shaw, Brian M; Kingsberg, Sheryl; Kopras, Elizabeth; Larkin, Lisa; McCormack, Francis X; Gupta, Nishant 2024. Hemoptysis Associated with Sexual Activity in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Akkenepally, Santhoshi V; Yombo, Dan J K; Yerubandi, Sanjana; Reddy, Geereddy Bhanuprakash; Deshpande, Deepak A; McCormack, Francis X; Madala, Satish K 2023. Interleukin 31 receptor α promotes smooth muscle cell contraction and airway hyperresponsiveness in asthma. Nature communications, 14 1, 8207Astrinidis, Aristotelis; Li, Chenggang; Zhang, Erik Y; Zhao, Xueheng; Zhao, Shuyang; Guo, Minzhe; Olatoke, Tasnim; Mattam, Ushodaya; Huang, Rong; Zhang, Alan G; Pitstick, Lori; Kopras, Elizabeth J; Gupta, Nishant; Jandarov, Roman; Smith, Eric P; Fugate, Elizabeth; Lindquist, Diana; Markiewski, Maciej M; Karbowniczek, Magdalena; Wikenheiser-Brokamp, Kathryn A; Setchell, Kenneth D R; McCormack, Francis X; Xu, Yan; Yu, Jane J 2023. Upregulation of acid ceramidase contributes to tumor progression in tuberous sclerosis complex. JCI insight, 8 9, Du, Yina; Guo, Minzhe; Wu, Yixin; Wagner, Andrew; Perl, Anne Karina; Wikenheiser-Brokamp, Kathryn; Yu, Jane; Gupta, Nishant; Kopras, Elizabeth; Krymskaya, Vera; Obraztsova, Kseniya; Tang, Yan; Kwiatkowski, David; Henske, Elizabeth P; McCormack, Francis; Xu, Yan 2023. Lymphangioleiomyomatosis (LAM) Cell Atlas. Thorax, 78 1, 85-87Evans, Jilly F; McCormack, Francis X; Sonenberg, Nahum; Krymskaya, Vera P 2023. Lost in translation: a neglected mTOR target for lymphangioleiomyomatosis. European respiratory review : an official journal of the European Respiratory Society, 32 169, Gajjala, Prathibha R; Singh, Priyanka; Odayar, Varshini; Ediga, Harshavardhana H; McCormack, Francis X; Madala, Satish K 2023. Wilms Tumor 1-Driven Fibroblast Activation and Subpleural Thickening in Idiopathic Pulmonary Fibrosis. International journal of molecular sciences, 24 3, Gupta, Nishant; Zhang, Bin; Zhou, Yuan; McCormack, Francis X; Ingledue, Rebecca; Robbins, Nathan; Kopras, Elizabeth J; McMahan, Susan; Singla, Abhishek; Swigris, Jeffrey; Cole, Adam G; Holz, Marina K 2023. Safety and Efficacy of Combined Resveratrol and Sirolimus in Lymphangioleiomyomatosis. Chest, 163 5, 1144-1155Harun, Nusrat; Gupta, Nishant; McCormack, Francis X; Macaluso, Maurizio 2023. Dynamic use of historical controls in clinical trials for rare disease research: A re-evaluation of the MILES trial. Clinical trials (London, England), 20 3, 223-234Hasegawa, Yoshihiro; Franks, Jennifer M; Tanaka, Yusuke; Uehara, Yasuaki; Read, David F; Williams, Claire; Srivatsan, Sanjay; Pitstick, Lori B; Nikolaidis, Nikolaos M; Shaver, Ciara M; Wu, Huixing; Gardner, Jason C; Osterburg, Andrew R; Yu, Jane J; Kopras, Elizabeth J; Teitelbaum, Steven L; Wikenheiser-Brokamp, Kathryn A; Trapnell, Cole; McCormack, Francis X 2023. Pulmonary osteoclast-like cells in silica induced pulmonary fibrosis. bioRxiv : the preprint server for biology, , Jose, Arun; Elwing, Jean M; Kawut, Steven M; Pauciulo, Michael W; Sherman, Kenneth E; Nichols, William C; Fallon, Michael B; McCormack, Francis X 2023. Human liver single nuclear RNA sequencing implicates BMPR2, GDF15, arginine, and estrogen in portopulmonary hypertension. Communications biology, 6 1, 826Lin, Hong T; Wikenheiser-Brokamp, Kathryn A; Udstuen, Gavin; Jones, Blaise; McCormack, Francis X 2023. Marked Improvement in Soft Tissue and CNS Manifestations of Adult Langerhans Cell Histiocytosis on Targeted MEK Inhibitor Therapy. Chest, 163 2, e53-e56McCormack, Fiona C; Hopley, Rachel C; Boath, Elizabeth H; Parry, Sian L; Roscoe, Suzie M; Stewart, Antony; Birch, Victoria A 2023. Exploring pregnant women's experiences of stopping smoking with an incentive scheme with 'enhanced' support: a qualitative study. Perspectives in public health, 143 5, 285-291Olatoke, Tasnim; Wagner, Andrew; Astrinidis, Aristotelis; Zhang, Erik Y; Guo, Minzhe; Zhang, Alan G; Mattam, Ushodaya; Kopras, Elizabeth J; Gupta, Nishant; Smith, Eric P; Karbowniczek, Magdalena; Markiewski, Maciej M; Wikenheiser-Brokamp, Kathryn A; Whitsett, Jeffrey A; McCormack, Francis X; Xu, Yan; Yu, Jane J 2023. Single-cell multiomic analysis identifies a HOX-PBX gene network regulating the survival of lymphangioleiomyomatosis cells. Science advances, 9 19, eadf8549Uehara, Yasuaki; Tanaka, Yusuke; Zhao, Shuyang; Nikolaidis, Nikolaos M; Pitstick, Lori B; Wu, Huixing; Yu, Jane J; Zhang, Erik; Hasegawa, Yoshihiro; Noel, John G; Gardner, Jason C; Kopras, Elizabeth J; Haffey, Wendy D; Greis, Kenneth D; Guo, Jinbang; Woods, Jason C; Wikenheiser-Brokamp, Kathryn A; Kyle, Jennifer E; Ansong, Charles; Teitelbaum, Steven L; Inoue, Yoshikazu; Altinişik, Göksel; Xu, Yan; McCormack, Francis X 2023. Insights into pulmonary phosphate homeostasis and osteoclastogenesis emerge from the study of pulmonary alveolar microlithiasis. Nature communications, 14 1, 1205Chernbumroong, Saisakul; Johnson, Janice; Gupta, Nishant; Miller, Suzanne; McCormack, Francis X; Garibaldi, Jonathan M; Johnson, Simon R 2021. Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis. The European respiratory journal, 57 6, Foley, M; Duffy, F; Skally, M; McCormack, F; Finn, C; O'Connor, M; Cafferkey, J; Thomas, T; Burns, K; Fitzpatrick, F; O'Connell, K; Smyth, E G; Humphreys, H 2021. Evolving epidemiology of carbapenemase-producing Enterobacterales: one hospital's infection prevention and control response over nine years. The Journal of hospital infection, 112 , 61-68McCarthy, Cormac; Gupta, Nishant; Johnson, Simon R; Yu, Jane J; McCormack, Francis X 2021. Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. The Lancet. Respiratory medicine, , Northrup, Hope; Aronow, Mary E; Bebin, E Martina; Bissler, John; Darling, Thomas N; de Vries, Petrus J; Frost, Michael D; Fuchs, Zoë; Gosnell, Elizabeth S; Gupta, Nishant; Jansen, Anna C; Jó?wiak, Sergiusz; Kingswood, J Chris; Knilans, Timothy K; McCormack, Francis X; Pounders, Ashley; Roberds, Steven L; Rodriguez-Buritica, David F; Roth, Jonathan; Sampson, Julian R; Sparagana, Steven; Thiele, Elizabeth Anne; Weiner, Howard L; Wheless, James W; Towbin, Alexander J; Krueger, Darcy A 2021. Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations. Pediatric neurology, 123 , 50-66Tian, Xinlun; Glass, Jennifer E; Kwiatkowski, David J; Towbin, Alexander J; Li, Yinan; Sund, Kristen L; Krueger, Darcy A; Franz, David N; McCormack, Francis X; Gupta, Nishant 2021. Lymphangioleiomyomatosis Association with Underlying Genotype in Patients with Tuberous Sclerosis Complex. Annals of the American Thoracic Society, 18 5, 815-819White, Mitchell R; Nikolaidis, Nikolaos M; McCormack, Francis; Crouch, Erika C; Hartshorn, Kevan L 2021. Viral Evasion of Innate Immune Defense: The Case of Resistance of Pandemic H1N1 Influenza A Virus to Human Mannose-Binding Proteins. Frontiers in microbiology, 12 , 774711Dellinger, Michael T; McCormack, Francis X 2020. The emergence of targetable MEKanisms in sporadic lymphatic disorders. EMBO molecular medicine, , e12822Guo, Minzhe; Yu, Jane J; Perl, Anne Karina; Wikenheiser-Brokamp, Kathryn A; Riccetti, Matt; Zhang, Erik Y; Sudha, Parvathi; Adam, Mike; Potter, Andrew; Kopras, Elizabeth J; Giannikou, Krinio; Potter, S Steven; Sherman, Sue; Hammes, Stephen R; Kwiatkowski, David J; Whitsett, Jeffrey A; McCormack, Francis X; Xu, Yan 2020. Single Cell Transcriptomic Analysis Identifies a Unique Pulmonary Lymphangioleiomyomatosis Cell. American journal of respiratory and critical care medicine, , Gupta, Nishant; Bleesing, Jack H; McCormack, Francis X 2020. Successful Response to Treatment with Sirolimus in Pulmonary Sarcoidosis. American journal of respiratory and critical care medicine, , Gupta, Nishant; Hagner, Miles; Wu, Huixing; Young, Lisa R; Palipana, Anushka; Szczesniak, Rhonda D; McCormack, Francis X 2020. Serum Vascular Endothelial Growth Factor-C as a Marker of Therapeutic Response to Sirolimus in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Jönsson, Åsa Lina M; Bendstrup, Elisabeth; Mogensen, Susie; Kopras, Elizabeth J; McCormack, Francis X; Campo, Ilaria; Mariani, Francesca; Escribano-Montaner, Amparo; Holm, Are M; Martinez-Colls, Maria Del Mar; Pintos-Morell, Guillem; Taillé, Camille; Crestani, Bruno; Hilberg, Ole; Hvarregaard Christensen, Jane; Simonsen, Ulf 2020. Eight novel variants in the SLC34A2 gene in pulmonary alveolar microlithiasis. The European respiratory journal, 55 2, Kosciuk, Patrick; Meyer, Cristopher; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2020. Pulmonary alveolar microlithiasis. European respiratory review : an official journal of the European Respiratory Society, 29 158, Krymskaya, Vera P; Courtwright, Andrew M; Fleck, Victoria; Dorgan, Daniel; Kotloff, Robert; McCormack, Francis X; Kreider, Maryl 2020. A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex. Respiratory medicine, 163 , 105898Liu, Huan; Osterburg, Andrew R; Flury, Jennifer; Swank, Zulma; McGraw, Dennis W; Gupta, Nishant; Wikenheiser-Brokamp, Kathryn A; Kumar, Ashish; Tazi, Abdellatif; Inoue, Yoshikazu; Hirose, Masaki; McCormack, Francis X; Borchers, Michael T 2020. MAPK mutations and cigarette smoke promote the pathogenesis of pulmonary Langerhans cell histiocytosis. JCI insight, 5 4, Poovelikunnel, Toney T; Duffy, Fionnuala; Puthussery, Thressia; Gangadharan, Shaini; McCormack, Fiona; Carpenter, Helen; Kizhakedath, Mary; Hawkshaw, Susan 2020. Clinically indicated replacement of peripheral vascular catheters: is it safe for patients? British journal of nursing (Mark Allen Publishing), 29 8, S4-S10Shaw, Brian M; Shaw, Steven D; McCormack, Francis X 2020. Pulmonary Alveolar Microlithiasis. Seminars in respiratory and critical care medicine, 41 2, 280-287Gupta, Nishant; Lee, Hye-Seung; Ryu, Jay H; Taveira-DaSilva, Angelo M; Beck, Gerald J; Lee, Jar-Chi; McCarthy, Kevin; Finlay, Geraldine A; Brown, Kevin K; Ruoss, Stephen J; Avila, Nilo A; Moss, Joel; McCormack, Francis X 2019. The NHLBI LAM Registry: Prognostic Physiologic and Radiologic Biomarkers Emerge From a 15-Year Prospective Longitudinal Analysis. Chest, 155 2, 288-296Gupta, Nishant; Lee, Hye-Seung; Young, Lisa R; Strange, Charlie; Moss, Joel; Singer, Lianne G; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Downey, Gregory P; Taveira-DaSilva, Angelo M; Krischer, Jeffrey P; Setchell, Kenneth; Trapnell, Bruce C; Inoue, Yoshikazu; McCormack, Francis X 2019. Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis. The European respiratory journal, 53 4, Hirose, Masaki; Matsumuro, Akiko; Arai, Toru; Sugimoto, Chikatoshi; Akira, Masanori; Kitaichi, Masanori; Young, Lisa R; McCormack, Francis X; Inoue, Yoshikazu 2019. Serum vascular endothelial growth factor-D as a diagnostic and therapeutic biomarker for lymphangioleiomyomatosis. PloS one, 14 2, e0212776McCarthy, Cormac; Bartholmai, Brian J; Woods, Jason C; McCormack, Francis X; Trapnell, Bruce C 2019. Automated Parenchymal Pattern Analysis of Treatment Responses in Pulmonary Alveolar Proteinosis. American journal of respiratory and critical care medicine, , McCarthy, Cormac; McCormack, Francis X 2019. Recurrent Sinusitis, Lower Limb Edema, and Nail Changes. Annals of the American Thoracic Society, 16 6, 752-755Nalian, Armen; Umstead, Todd M; Yang, Ching-Hui; Silveyra, Patricia; Thomas, Neal J; Floros, Joanna; McCormack, Francis X; Chroneos, Zissis C 2019. Structural and Functional Determinants of Rodent and Human Surfactant Protein A: A Synthesis of Binding and Computational Data. Frontiers in immunology, 10 , 2613Walkup, Laura L; Roach, David J; Hall, Chase S; Gupta, Nishant; Thomen, Robert P; Cleveland, Zackary I; McCormack, Francis X; Woods, Jason C 2019. Cyst Ventilation Heterogeneity and Alveolar Airspace Dilation as Early Disease Markers in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 16 8, 1008-1016Zak, Sara; Mokhallati, Nadine; Su, Weiji; McCormack, Francis X; Franz, David N; Mays, Maxwell; Krueger, Darcy A; Szczesniak, Rhonda D; Gupta, Nishant 2019. Lymphangioleiomyomatosis Mortality in Patients with Tuberous Sclerosis Complex. Annals of the American Thoracic Society, 16 4, 509-512Gupta, Nishant; Colby, Thomas V; Meyer, Cristopher A; McCormack, Francis X; Wikenheiser-Brokamp, Kathryn A 2018. Smoking-Related Diffuse Cystic Lung Disease. Chest, 154 2, e31-e35Gupta, Nishant; Johnson, Simon R; Moss, Joel; McCormack, Francis X 2018. Reply to Yanagisawa: Treatment of Pulmonary Lymphangioleiomyomatosis during Pregnancy. American journal of respiratory and critical care medicine, 197 11, 1507-1508Liu, Huan; Osterburg, Andrew R; Flury, Jennifer; Huang, Shuo; McCormack, Francis X; Cormier, Stephania A; Borchers, Michael T 2018. NKG2D Regulation of Lung Pathology and Dendritic Cell Function Following Respiratory Syncytial Virus Infection. The Journal of infectious diseases, 218 11, 1822-1832Miller, Suzanne; Coveney, Clare; Johnson, Janice; Farmaki, Aliki-Eleni; Gupta, Nishant; Tobin, Martin D; Wain, Louise V; McCormack, Francis X; Boocock, David J; Johnson, Simon R 2018. The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis. The European respiratory journal, 52 5, Feemster, Laura C; Lyons, Patrick G; Chatterjee, Rohini S; Kidambi, Pranav; McCormack, Francis X; Moss, Joel; Wilson, Kevin C; Thomson, Carey C 2017. Summary for Clinicians: Lymphangioleiomyomatosis Diagnosis and Management Clinical Practice Guideline. Annals of the American Thoracic Society, 14 7, 1073-1075Gupta, Nishant; Finlay, Geraldine A; Kotloff, Robert M; Strange, Charlie; Wilson, Kevin C; Young, Lisa R; Taveira-DaSilva, Angelo M; Johnson, Simon R; Cottin, Vincent; Sahn, Steven A; Ryu, Jay H; Seyama, Kuniaki; Inoue, Yoshikazu; Downey, Gregory P; Han, MeiLan K; Colby, Thomas V; Wikenheiser-Brokamp, Kathryn A; Meyer, Cristopher A; Smith, Karen; Moss, Joel; McCormack, Francis X 2017. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. American journal of respiratory and critical care medicine, 196 10, 1337-1348Gupta, Nishant; Kopras, Elizabeth J; Henske, Elizabeth P; James, Laura E; El-Chemaly, Souheil; Veeraraghavan, Srihari; Drake, Matthew G; McCormack, Francis X 2017. Spontaneous Pneumothoraces in Patients with Birt-Hogg-Dubé Syndrome. Annals of the American Thoracic Society, 14 5, 706-713Gupta, Nishant; Langenderfer, Dale; McCormack, Francis X; Schauer, Daniel P; Eckman, Mark H 2017. Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective. Annals of the American Thoracic Society, 14 1, 17-25Han, MeiLan K; Tyburczy, Magdalena E; Darling, Thomas N; Kazerooni, Ella A; Myers, Jeffrey L; McCormack, Francis X; Moss, Joel; Kwiatkowski, David J 2017. Apparent Sporadic Lymphangioleiomyomatosis (LAM) in a Man Due to Extreme Mosaicism for a TSC2 Mutation. Annals of the American Thoracic Society, , Kristof, Arnold S; Petrof, Basil J; Hamid, Qutayba; Kolb, Martin; Landry, Jennifer S; MacKenzie, Alex; McCormack, Francis X; Murawski, Inga J; Moss, Joel; Rauch, Frank; Rosas, Ivan O; Shapiro, Adam J; Smith, Benjamin M; Thomas, David Y; Trapnell, Bruce C; Young, Lisa R; Zariwala, Maimoona A 2017. An Official American Thoracic Society Workshop Report: Translational Research in Rare Respiratory Diseases. Annals of the American Thoracic Society, 14 8, 1239-1247Krymskaya, Vera P; McCormack, Francis X 2017. Lymphangioleiomyomatosis: A Monogenic Model of Malignancy. Annual review of medicine, 68 , 69-83Lee, Lynn H; Gasilina, Anjelika; Roychoudhury, Jayeeta; Clark, Jason; McCormack, Francis X; Pressey, Joseph; Grimley, Michael S; Lorsbach, Robert; Ali, Siraj; Bailey, Mark; Stephens, Philip; Ross, Jeffrey S; Miller, Vincent A; Nassar, Nicolas N; Kumar, Ashish R 2017. Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes. JCI insight, 2 3, e89473Liu, Huan; Jakubzick, Claudia; Osterburg, Andrew R; Nelson, Rebecca L; Gupta, Nishant; McCormack, Francis X; Borchers, Michael T 2017. Dendritic Cell Trafficking and Function in Rare Lung Diseases. American journal of respiratory cell and molecular biology, , Lu, Calvin; Lee, Hye-Seung; Pappas, George P; Dilling, Daniel F; Burger, Charles D; Shifren, Adrian; Veeraraghavan, Srihari; Chapman, Jeffrey T; Parambil, Joseph; Ruoss, Stephen J; Young, Lisa R; Hammes, Stephen R; Kopras, Elizabeth J; Roads, Tammy; Krischer, Jeffrey P; McCormack, Francis X 2017. A Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 14 6, 919-928McCarthy, Cormac; Lara Gallego, Beatriz; Trapnell, Bruce C; McCormack, Francis X 2017. Epidemiology of Rare Lung Diseases: The Challenges and Opportunities to Improve Research and Knowledge. Advances in experimental medicine and biology, 1031 , 419-442McCormack, Francis X; Gupta, Nishant; Finlay, Geraldine; Young, Lisa R; Johnson, Simon; Cottin, Vincent; Wilson, Kevin; Moss, Joel 2017. Reply to Official ATS/JRS Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. American journal of respiratory and critical care medicine, , Nikolaidis, Nikolaos M; Noel, John G; Pitstick, Lori B; Gardner, Jason C; Uehara, Yasuaki; Wu, Huixing; Saito, Atsushi; Lewnard, Kara E; Liu, Huan; White, Mitchell R; Hartshorn, Kevan L; McCormack, Francis X 2017. Mitogenic stimulation accelerates influenza-induced mortality by increasing susceptibility of alveolar type II cells to infection. Proceedings of the National Academy of Sciences of the United States of America, 114 32, E6613-E6622Noel, John G; Ramser, Benjamin J; Cancelas, Jose A; McCormack, Francis X; Gardner, Jason C 2017. Thermal injury of the skin induces G-CSF-dependent attenuation of EPO-mediated STAT signaling and erythroid differentiation arrest in mice. Experimental hematology, , Rynkiewicz, Michael J; Wu, Huixing; Cafarella, Tanya R; Nikolaidis, Nikolaos M; Head, James F; Seaton, Barbara A; McCormack, Francis X 2017. Differential Ligand Binding Specificities of the Pulmonary Collectins Are Determined by the Conformational Freedom of a Surface Loop. Biochemistry, 56 31, 4095-4105Sontake, Vishwaraj; Wang, Yunguan; Kasam, Rajesh K; Sinner, Debora; Reddy, Geereddy B; Naren, Anjaparavanda P; McCormack, Francis X; White, Eric S; Jegga, Anil G; Madala, Satish K 2017. Hsp90 regulation of fibroblast activation in pulmonary fibrosis. JCI insight, 2 4, e91454Wang, Yunguan; Yella, Jaswanth; Chen, Jing; McCormack, Francis X; Madala, Satish K; Jegga, Anil G 2017. Unsupervised gene expression analyses identify IPF-severity correlated signatures, associated genes and biomarkers. BMC pulmonary medicine, 17 1, 133Argula, Rahul G; Kokosi, Maria; Lo, Pechin; Kim, Hyun J; Ravenel, James G; Meyer, Cristopher; Goldin, Jonathan; Lee, Hye-Seung; Strange, Charlie; McCormack, Francis X 2016. A Novel Quantitative Computed Tomographic Analysis Suggests How Sirolimus Stabilizes Progressive Air Trapping in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 13 3, 342-9Gardner, Jason C; Wu, Huixing; Noel, John G; Ramser, Benjamin J; Pitstick, Lori; Saito, Atsushi; Nikolaidis, Nikolaos M; McCormack, Francis X 2016. Keratinocyte growth factor supports pulmonary innate immune defense through maintenance of alveolar antimicrobial protein levels and macrophage function. American journal of physiology. Lung cellular and molecular physiology, 310 9, L868-79Goh, Boon Chong; Wu, Huixing; Rynkiewicz, Michael J; Schulten, Klaus; Seaton, Barbara A; McCormack, Francis X 2016. Elucidation of Lipid Binding Sites on Lung Surfactant Protein A Using X-ray Crystallography, Mutagenesis, and Molecular Dynamics Simulations. Biochemistry, 55 26, 3692-701Gupta, Nishant; Wikenheiser-Brokamp, Kathryn A; Fischer, Aryeh; McCormack, Francis X 2016. Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren Syndrome. Annals of the American Thoracic Society, 13 3, 371-5Itkin, Maxim G; McCormack, Francis X; Dori, Yoav 2016. Diagnosis and Treatment of Lymphatic Plastic Bronchitis in Adults Using Advanced Lymphatic Imaging and Percutaneous Embolization. Annals of the American Thoracic Society, 13 10, 1689-1696Itkin, Maxim; McCormack, Francis X 2016. Nonmalignant Adult Thoracic Lymphatic Disorders. Clinics in chest medicine, 37 3, 409-20Kotloff, Robert M; McCormack, Francis X 2016. Rare Lung Diseases: Occasionally the "Horse" Has Stripes. Clinics in chest medicine, 37 3, xv-xviMcCormack, Francis X; Gupta, Nishant; Finlay, Geraldine R; Young, Lisa R; Taveira-DaSilva, Angelo M; Glasgow, Connie G; Steagall, Wendy K; Johnson, Simon R; Sahn, Steven A; Ryu, Jay H; Strange, Charlie; Seyama, Kuniaki; Sullivan, Eugene J; Kotloff, Robert M; Downey, Gregory P; Chapman, Jeffrey T; Han, MeiLan K; D'Armiento, Jeanine M; Inoue, Yoshikazu; Henske, Elizabeth P; Bissler, John J; Colby, Thomas V; Kinder, Brent W; Wikenheiser-Brokamp, Kathryn A; Brown, Kevin K; Cordier, Jean F; Meyer, Cristopher; Cottin, Vincent; Brozek, Jan L; Smith, Karen; Wilson, Kevin C; Moss, Joel 2016. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. American journal of respiratory and critical care medicine, 194 6, 748-61Osterburg, Andrew R; Nelson, Rebecca L; Yaniv, Benyamin Z; Foot, Rachel; Donica, Walter R F; Nashu, Madison A; Liu, Huan; Wikenheiser-Brokamp, Kathryn A; Moss, Joel; Gupta, Nishant; McCormack, Francis X; Borchers, Michael T 2016. NK cell activating receptor ligand expression in lymphangioleiomyomatosis is associated with lung function decline. JCI insight, 1 16, e87270Saito, Atsushi; McCormack, Francis X 2016. Pulmonary Alveolar Microlithiasis. Clinics in chest medicine, 37 3, 441-8Soleimani, Manoocher; Barone, Sharon; Xu, Jie; Alshahrani, Saeed; Brooks, Marybeth; McCormack, Francis X; Smith, Roger D; Zahedi, Kamyar 2016. Prostaglandin-E2 Mediated Increase in Calcium and Phosphate Excretion in a Mouse Model of Distal Nephron Salt Wasting. PloS one, 11 7, e0159804Takada, Toshinori; Mikami, Ayako; Kitamura, Nobutaka; Seyama, Kuniaki; Inoue, Yoshikazu; Nagai, Katsura; Suzuki, Masaru; Moriyama, Hiroshi; Akasaka, Keiichi; Tazawa, Ryushi; Hirai, Toyohiro; Mishima, Michiaki; Hayashida, Mie; Hirose, Masaki; Sugimoto, Chikatoshi; Arai, Toru; Hattori, Noboru; Watanabe, Kentaro; Tamada, Tsutomu; Yoshizawa, Hirohisa; Akazawa, Kohei; Tanaka, Takahiro; Yagi, Keita; Young, Lisa R; McCormack, Francis X; Nakata, Koh 2016. Efficacy and Safety of Long-term Sirolimus Therapy for Asian Patients with Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Goldberg, Hilary J; Harari, Sergio; Cottin, Vincent; Rosas, Ivan O; Peters, Elizabeth; Biswal, Shibadas; Cheng, Yi; Khindri, Sanjeev; Kovarik, John M; Ma, Shenglin; McCormack, Francis X; Henske, Elizabeth Petri 2015. Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study. The European respiratory journal, 46 3, 783-94Gupta, Nishant; Han, MeiLan K; McCormack, Francis X 2015. Regional Sparing in an Oligemic Lung Segment Supports Hematogenous Spread as a Pathogenic Mechanism in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 12 8, 1247-8Gupta, Nishant; Meraj, Riffat; Tanase, Daniel; James, Laura E; Seyama, Kuniaki; Lynch, David A; Akira, Masanori; Meyer, Cristopher A; Ruoss, Stephen J; Burger, Charles D; Young, Lisa R; Almoosa, Khalid F; Veeraraghavan, Srihari; Barker, Alan F; Lee, Augustine S; Dilling, Daniel F; Inoue, Yoshikazu; Cudzilo, Corey J; Zafar, Muhammad A; McCormack, Francis X 2015. Accuracy of chest high-resolution computed tomography in diagnosing diffuse cystic lung diseases. The European respiratory journal, 46 4, 1196-9Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2015. Diffuse Cystic Lung Disease. Part II. American journal of respiratory and critical care medicine, 192 1, 17-29Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2015. Diffuse Cystic Lung Disease. Part I. American journal of respiratory and critical care medicine, 191 12, 1354-66Gupta, Rajeev; McCormack, Francis X 2015. Response. Lymphology, 48 1, 52Mulhall, Aaron M; McCormack, Francis X 2015. A 25-year-old man with fatigue, dyspnea, and pulmonary cysts. Annals of the American Thoracic Society, 12 1, 120-3Pasula, Rajamouli; Azad, Abul K; Gardner, Jason C; Schlesinger, Larry S; McCormack, Francis X 2015. Keratinocyte growth factor administration attenuates murine pulmonary mycobacterium tuberculosis infection through granulocyte-macrophage colony-stimulating factor (GM-CSF)-dependent macrophage activation and phagolysosome fusion. The Journal of biological chemistry, 290 11, 7151-9Quintanilla, Hector D; Liu, Yuying; Fatheree, Nicole Y; Atkins, Constance L; Hashmi, Syed S; Floros, Joanna; McCormack, Francis X; Rhoads, Jon Marc; Alcorn, Joseph L 2015. Oral administration of surfactant protein-a reduces pathology in an experimental model of necrotizing enterocolitis. Journal of pediatric gastroenterology and nutrition, 60 5, 613-20Saito, Atsushi; Nikolaidis, Nikolaos M; Amlal, Hassane; Uehara, Yasuaki; Gardner, Jason C; LaSance, Kathleen; Pitstick, Lori B; Bridges, James P; Wikenheiser-Brokamp, Kathryn A; McGraw, Dennis W; Woods, Jason C; Sabbagh, Yves; Schiavi, Susan C; Altini?ik, Göksel; Jakopovi?, Marko; Inoue, Yoshikazu; McCormack, Francis X 2015. Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment. Science translational medicine, 7 313, 313ra181Yang, Linlin; Carrillo, Marykate; Wu, Yuchieh M; DiAngelo, Susan L; Silveyra, Patricia; Umstead, Todd M; Halstead, E Scott; Davies, Michael L; Hu, Sanmei; Floros, Joanna; McCormack, Francis X; Christensen, Neil D; Chroneos, Zissis C 2015. SP-R210 (Myo18A) Isoforms as Intrinsic Modulators of Macrophage Priming and Activation. PloS one, 10 5, e0126576Cundiff, William B; McCormack, Francis X; Wikenheiser-Brokamp, Kathryn; Starnes, Sandra; Kotloff, Robert; Benzaquen, Sadia 2014. Successful management of a chronic, refractory bronchopleural fistula with endobronchial valves followed by talc pleurodesis. American journal of respiratory and critical care medicine, 189 4, 490-1Gardner, Jason C; Noel, John G; Nikolaidis, Nikolaos M; Karns, Rebekah; Aronow, Bruce J; Ogle, Cora K; McCormack, Francis X 2014. G-CSF drives a posttraumatic immune program that protects the host from infection. Journal of immunology (Baltimore, Md. : 1950), 192 5, 2405-17Gupta, R; Kitaichi, M; Inoue, Y; Kotloff, R; McCormack, F X 2014. Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology, 47 3, 106-17Gupta, R; Kitaichi, M; Inoue, Y; Kotloff, R; McCormack, F X 2014. Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology, 47 3, 106-17McCormack, Francis X 2014. Chronic sirolimus therapy for lymphangioleiomyomatosis. American journal of respiratory and critical care medicine, 190 12, 1332-3Nikolaidis, Nikolaos M; White, Mitchell R; Allen, Kimberly; Tripathi, Shweta; Qi, Li; McDonald, Barbara; Taubenberger, Jeffery; Seaton, Barbara A; McCormack, Francis X; Crouch, Erika C; Hartshorn, Kevan L 2014. Mutations flanking the carbohydrate binding site of surfactant protein D confer antiviral activity for pandemic influenza A viruses. American journal of physiology. Lung cellular and molecular physiology, 306 11, L1036-44Safi, Fadi; Gupta, Anita; Adams, Denise; Anandan, Vasuki; McCormack, Francis X; Assaly, Ragheb 2014. Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. Annals of the American Thoracic Society, 11 1, 92-5Cudzilo, Corey J; Szczesniak, Rhonda D; Brody, Alan S; Rattan, Mantosh S; Krueger, Darcy A; Bissler, John J; Franz, David N; McCormack, Francis X; Young, Lisa R 2013. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest, 144 2, 578-85Czyzyk-Krzeska, Maria F; McCormack, Francis X 2013. Birt-Hogg-Dubé syndrome. Familial cancer, 12 3, 355-6Gupta, Nishant; McCormack, Francis X 2013. Pulmonary alveolar microlithiasis. American journal of respiratory and critical care medicine, 188 7, e11-2Gupta, Nishant; Seyama, Kuniaki; McCormack, Francis X 2013. Pulmonary manifestations of Birt-Hogg-Dubé syndrome. Familial cancer, 12 3, 387-96Krueger, Darcy A; Northrup, Hope 2013. Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatric neurology, 49 4, 255-65McCormack, Francis X; Travis, William D; Colby, Thomas V; Henske, Elizabeth P; Moss, Joel 2013. Reply: Characterization of lymphangioleiomyomatosis as a neoplastic disease. American journal of respiratory and critical care medicine, 188 3, 398-9McCormack, Francis X; Wilmott, Clay; Young, Lisa R; Wikenheiser-Brokamp, Kathryn 2013. An atoll variant that mocks. American journal of respiratory and critical care medicine, 188 1, 110Northrup, Hope; Krueger, Darcy A 2013. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 Iinternational Tuberous Sclerosis Complex Consensus Conference. Pediatric neurology, 49 4, 243-54Oprescu, N; McCormack, F X; Byrnes, S; Kinder, B W 2013. Clinical predictors of mortality and cause of death in lymphangioleiomyomatosis: a population-based registry. Lung, 191 1, 35-42Safi, Fadi; Gupta, Anita; Adams, Denise; Anandan, Vasuki; McCormack, Francis X; Assaly, Ragheb 2013. Kaposiform Lymphangiomatosis, a Newly Characterized Vascular Anomaly Presenting with Hemoptysis in an Adult Woman. Annals of the American Thoracic Society, , Swigris, Jeffrey J; Lee, Hye-Seung; Cohen, Marsha; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Young, Lisa R; McCormack, Francis X 2013. St. George's Respiratory Questionnaire has longitudinal construct validity in lymphangioleiomyomatosis. Chest, 143 6, 1671-8Young, Lisa R; Lee, Hye-Seung; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Strange, Charlie; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Downey, Gregory P; Swigris, Jeffrey J; Taveira-Dasilva, Angelo M; Krischer, Jeffrey P; Trapnell, Bruce C; McCormack, Francis X 2013. Serum VEGF-D concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. The lancet. Respiratory medicine, 1 6, 445-452Zafar, Muhammad A; McCormack, Francis X; Rahman, Shahid; Tencza, Christian; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; Shizukuda, Yukitaka; Elwing, Jean M 2013. Pulmonary vascular shunts in exercise-intolerant patients with lymphangioleiomyomatosis. American journal of respiratory and critical care medicine, 188 9, 1167-70Henske, Elizabeth P; McCormack, Francis X 2012. Lymphangioleiomyomatosis - a wolf in sheep's clothing. The Journal of clinical investigation, 122 11, 3807-16McCormack, Francis X; Travis, William D; Colby, Thomas V; Henske, Elizabeth P; Moss, Joel 2012. Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. American journal of respiratory and critical care medicine, 186 12, 1210-2Meraj, Riffat; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; Byrnes, Sue; McCormack, Francis X 2012. Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis. Frontiers of medicine, 6 4, 395-405Meraj, Riffat; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; McCormack, Francis X 2012. Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Seminars in respiratory and critical care medicine, 33 5, 486-97Young, Lisa R; Gulleman, Peter M; Bridges, James P; Weaver, Timothy E; Deutsch, Gail H; Blackwell, Timothy S; McCormack, Francis X 2012. The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome. American journal of respiratory and critical care medicine, 186 10, 1014-24Crouch, Erika; Nikolaidis, Nikolaos; McCormack, Francis X; McDonald, Barbara; Allen, Kimberly; Rynkiewicz, Michael J; Cafarella, Tanya M; White, Mitchell; Lewnard, Kara; Leymarie, Nancy; Zaia, Joseph; Seaton, Barbara A; Hartshorn, Kevan L 2011. Mutagenesis of surfactant protein D informed by evolution and x-ray crystallography enhances defenses against influenza A virus in vivo. The Journal of biological chemistry, 286 47, 40681-92Hagaman, Jared T; Panos, Ralph J; McCormack, Francis X; Thakar, Charuhas V; Wikenheiser-Brokamp, Kathryn A; Shipley, Ralph T; Kinder, Brent W 2011. Vitamin D deficiency and reduced lung function in connective tissue-associated interstitial lung diseases. Chest, 139 2, 353-60McCormack, Francis X; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Strange, Charlie; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Young, Lisa R; Kinder, Brent W; Downey, Gregory P; Sullivan, Eugene J; Colby, Thomas V; McKay, Roy T; Cohen, Marsha M; Korbee, Leslie; Taveira-DaSilva, Angelo M; Lee, Hye-Seung; Krischer, Jeffrey P; Trapnell, Bruce C 2011. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. The New England journal of medicine, 364 17, 1595-606Seaman, Danielle M; Meyer, Cristopher A; Gilman, Matthew D; McCormack, Francis X 2011. Diffuse Cystic Lung Disease at High-Resolution CT. AJR. American journal of roentgenology, 196 6, 1305-11Shang, Feifei; Rynkiewicz, Michael J; McCormack, Francis X; Wu, Huixing; Cafarella, Tanya M; Head, James F; Seaton, Barbara A 2011. Crystallographic complexes of surfactant protein A and carbohydrates reveal ligand-induced conformational change. The Journal of biological chemistry, 286 1, 757-65Wu, Huixing; Suzuki, Takuji; Carey, Brenna; Trapnell, Bruce C; McCormack, Francis X 2011. Keratinocyte growth factor augments pulmonary innate immunity through epithelium-driven, GM-CSF-dependent paracrine activation of alveolar macrophages. The Journal of biological chemistry, 286 17, 14932-40Franz, D N; Bissler, J J; McCormack, F X 2010. Tuberous sclerosis complex: neurological, renal and pulmonary manifestations. Neuropediatrics, 41 5, 199-208Hagaman, Jared T; Ferguson, Bryce; Meyer, Cris; Wang, Jiang; McCormack, Francis X; Nath, Amaresh 2010. Endobronchial Carcinoid Tumor in an HIV-infected Patient. Journal of bronchology & interventional pulmonology, 17 2, 174-6Hagaman, Jared T; Schauer, Daniel P; McCormack, Francis X; Kinder, Brent W 2010. Screening for lymphangioleiomyomatosis by high-resolution computed tomography in young, nonsmoking women presenting with spontaneous pneumothorax is cost-effective. American journal of respiratory and critical care medicine, 181 12, 1376-82Johnson, S R; Cordier, J F; Lazor, R; Cottin, V; Costabel, U; Harari, S; Reynaud-Gaubert, M; Boehler, A; Brauner, M; Popper, H; Bonetti, F; Kingswood, C 2010. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. The European respiratory journal, 35 1, 14-26Kinder, Brent W; Sherman, A C; Young, L R; Hagaman, J T; Oprescu, N; Byrnes, S; McCormack, Francis X 2010. Predictors for clinical trial participation in the rare lung disease lymphangioleiomyomatosis. Respiratory medicine, 104 4, 578-83Kinder, Brent; McCormack, Francis X 2010. Clinical trials for rare lung diseases: lessons from lymphangioleiomyomatosis. Lymphatic research and biology, 8 1, 71-9Seaton, Barbara A; Crouch, Erika C; McCormack, Francis X; Head, James F; Hartshorn, Kevan L; Mendelsohn, Richard 2010. Review: Structural determinants of pattern recognition by lung collectins. Innate immunity, 16 3, 143-50Young, Lisa R; Vandyke, Rhonda; Gulleman, Peter M; Inoue, Yoshikazu; Brown, Kevin K; Schmidt, Laura S; Linehan, W Marston; Hajjar, Fuad; Kinder, Brent W; Trapnell, Bruce C; Bissler, John J; Franz, David N; McCormack, Francis X 2010. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest, 138 3, 674-81Gil, Malgorzata; McCormack, Francis X; Levine, Ann Marie 2009. Surfactant protein A modulates cell surface expression of CR3 on alveolar macrophages and enhances CR3-mediated phagocytosis. The Journal of biological chemistry, 284 12, 7495-504Kinder, Brent W; Brown, Kevin K; McCormack, Francis X; Ix, Joachim H; Kervitsky, Alma; Schwarz, Marvin I; King, Talmadge E 2009. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest, 135 6, 1557-63Schmithorst, Vincent J; Altes, Talissa A; Young, Lisa R; Franz, David N; Bissler, John J; McCormack, Francis X; Dardzinski, Bernard J; Brody, Alan S 2009. Automated algorithm for quantifying the extent of cystic change on volumetric chest CT: initial results in Lymphangioleiomyomatosis. AJR. American journal of roentgenology, 192 4, 1037-44Young, Lisa R; Franz, David N; Nagarkatte, Preeti; Fletcher, Christopher D M; Wikenheiser-Brokamp, Kathryn A; Galsky, Matthew D; Corbridge, Thomas C; Lam, Anna P; Gelfand, Michael J; McCormack, Francis X 2009. Utility of [18F]2-fluoro-2-deoxyglucose-PET in sporadic and tuberous sclerosis-associated lymphangioleiomyomatosis. Chest, 136 3, 926-33Ali, Naeem A; O'Brien, James M; Hoffmann, Stephen P; Phillips, Gary; Garland, Allan; Finley, James C W; Almoosa, Khalid; Hejal, Rana; Wolf, Karen M; Lemeshow, Stanley; Connors, Alfred F; Marsh, Clay B 2008. Acquired weakness, handgrip strength, and mortality in critically ill patients. American journal of respiratory and critical care medicine, 178 3, 261-8Bissler, John J; McCormack, Francis X; Young, Lisa R; Elwing, Jean M; Chuck, Gail; Leonard, Jennifer M; Schmithorst, Vincent J; Laor, Tal; Brody, Alan S; Bean, Judy; Salisbury, Shelia; Franz, David N 2008. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. The New England journal of medicine, 358 2, 140-51McCormack, Francis X 2008. Lymphangioleiomyomatosis: a clinical update. Chest, 133 2, 507-16Young, Lisa R; Inoue, Yoshikazu; McCormack, Francis X 2008. Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis. The New England journal of medicine, 358 2, 199-200Byrnes, Abigail B; McCormack, Francis X; Diers, Tiffiny; Jazieh, Abdul-Rahman 2007. The resident scholar program: a research training opportunity for internal medicine house staff. Journal of cancer education : the official journal of the American Association for Cancer Education, 22 1, 47-9Juvet, Stephen C; McCormack, Francis X; Kwiatkowski, David J; Downey, Gregory P 2007. Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphans. American journal of respiratory cell and molecular biology, 36 4, 398-408McCormack, Francis X; Moss, Joel 2007. S-LAM in a man? American journal of respiratory and critical care medicine, 176 1, 3-5Pollock-BarZiv, Stacey; Cohen, Marsha M; Downey, Gregory P; Johnson, Simon R; Sullivan, Eugene; McCormack, Francis X 2007. Air travel in women with lymphangioleiomyomatosis. Thorax, 62 2, 176-80Young, Lisa R; Pasula, Rajamouli; Gulleman, Peter M; Deutsch, Gail H; McCormack, Francis X 2007. Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosis. American journal of respiratory cell and molecular biology, 37 1, 67-74Zhang, Shiping; McCormack, Francis X; Levesque, Roger C; O'Toole, George A; Lau, Gee W 2007. The flagellum of Pseudomonas aeruginosa is required for resistance to clearance by surfactant protein A. PloS one, 2 6, e564Almoosa, Khalid F; McCormack, Francis X; Sahn, Steven A 2006. Pleural disease in lymphangioleiomyomatosis. Clinics in chest medicine, 27 2, 355-68Almoosa, Khalid F; Ryu, Jay H; Mendez, Jose; Huggins, J Terrill; Young, Lisa R; Sullivan, Eugene J; Maurer, Janet; McCormack, Francis X; Sahn, Steven A 2006. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest, 129 5, 1274-81Kingma, Paul S; Zhang, Liqian; Ikegami, Machiko; Hartshorn, Kevan; McCormack, Francis X; Whitsett, Jeffrey A 2006. Correction of pulmonary abnormalities in Sftpd-/- mice requires the collagenous domain of surfactant protein D. The Journal of biological chemistry, 281 34, 24496-505Kuzmenko, Alexander I; Wu, Huixing; McCormack, Francis X 2006. Pulmonary collectins selectively permeabilize model bacterial membranes containing rough lipopolysaccharide. Biochemistry, 45 8, 2679-85McCormack, Francis X 2006. Lymphangioleiomyomatosis. MedGenMed : Medscape general medicine, 8 1, 15McCormack, Francis X 2006. New concepts in collectin-mediated host defense at the air-liquid interface of the lung. Respirology (Carlton, Vic.), 11 Suppl , S7-10Ryan, Marnie A; Akinbi, Henry T; Serrano, Alicia G; Perez-Gil, Jesus; Wu, Huixing; McCormack, Francis X; Weaver, Timothy E 2006. Antimicrobial activity of native and synthetic surfactant protein B peptides. Journal of immunology (Baltimore, Md. : 1950), 176 1, 416-25Ryu, Jay H; Moss, Joel; Beck, Gerald J; Lee, Jar-Chi; Brown, Kevin K; Chapman, Jeffrey T; Finlay, Geraldine A; Olson, Eric J; Ruoss, Stephen J; Maurer, Janet R; Raffin, Thomas A; Peavy, Hannah H; McCarthy, Kevin; Taveira-Dasilva, Angelo; McCormack, Francis X; Avila, Nilo A; Decastro, Rosamma M; Jacobs, Susan S; Stylianou, Mario; Fanburg, Barry L 2006. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. American journal of respiratory and critical care medicine, 173 1, 105-11Schlosser, A; Thomsen, T; Shipley, J M; Hein, P W; Brasch, F; Tornøe, I; Nielsen, O; Skjødt, K; Palaniyar, N; Steinhilber, W; McCormack, F X; Holmskov, U 2006. Microfibril-associated protein 4 binds to surfactant protein A (SP-A) and colocalizes with SP-A in the extracellular matrix of the lung. Scandinavian journal of immunology, 64 2, 104-16Young, Lisa R; Almoosa, Khalid F; Pollock-Barziv, Stacey; Coutinho, Meg; McCormack, Francis X; Sahn, Steven A 2006. Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis. Chest, 129 5, 1267-73Young, Lisa R; Borchers, Michael T; Allen, Holly L; Gibbons, Reta S; McCormack, Francis X 2006. Lung-restricted macrophage activation in the pearl mouse model of Hermansky-Pudlak syndrome. Journal of immunology (Baltimore, Md. : 1950), 176 7, 4361-8Zhang, Liqian; Ikegami, Machiko; Korfhagen, Thomas R; McCormack, Francis X; Yoshida, Mitsuhiro; Senior, Robert M; Shipley, J Michael; Shapiro, Steven D; Whitsett, Jeffrey A 2006. Neither SP-A nor NH2-terminal domains of SP-A can substitute for SP-D in regulation of alveolar homeostasis. American journal of physiology. Lung cellular and molecular physiology, 291 2, L181-90Beharka, Alison A; Crowther, Joy E; McCormack, Francis X; Denning, Gerene M; Lees, Jason; Tibesar, Eric; Schlesinger, Larry S 2005. Pulmonary surfactant protein A activates a phosphatidylinositol 3-kinase/calcium signal transduction pathway in human macrophages: participation in the up-regulation of mannose receptor activity. Journal of immunology (Baltimore, Md. : 1950), 175 4, 2227-36Kuzmenko, Alexander I; Wu, Huixing; Wan, Sijue; McCormack, Francis X 2005. Surfactant protein A is a principal and oxidation-sensitive microbial permeabilizing factor in the alveolar lining fluid. The Journal of biological chemistry, 280 27, 25913-9Zhang, Shiping; Chen, Yi; Potvin, Eric; Sanschagrin, Francois; Levesque, Roger C; McCormack, Francis X; Lau, Gee W 2005. Comparative signature-tagged mutagenesis identifies Pseudomonas factors conferring resistance to the pulmonary collectin SP-A. PLoS pathogens, 1 3, 259-68Crowther, Joy E; Kutala, Vijay Kumar; Kuppusamy, Periannan; Ferguson, J Scott; Beharka, Alison A; Zweier, Jay L; McCormack, Francis X; Schlesinger, Larry S 2004. Pulmonary surfactant protein a inhibits macrophage reactive oxygen intermediate production in response to stimuli by reducing NADPH oxidase activity. Journal of immunology (Baltimore, Md. : 1950), 172 11, 6866-74Kuzmenko, A I; Wu, H; Bridges, J P; McCormack, F X 2004. Surfactant lipid peroxidation damages surfactant protein A and inhibits interactions with phospholipid vesicles. Journal of lipid research, 45 6, 1061-8Schaeffer, Lyndsay M; McCormack, Francis X; Wu, Huixing; Weiss, Alison A 2004. Interactions of pulmonary collectins with Bordetella bronchiseptica and Bordetella pertussis lipopolysaccharide elucidate the structural basis of their antimicrobial activities. Infection and immunity, 72 12, 7124-30Schaeffer, Lyndsay M; McCormack, Francis X; Wu, Huixing; Weiss, Alison A 2004. Bordetella pertussis lipopolysaccharide resists the bactericidal effects of pulmonary surfactant protein A. Journal of immunology (Baltimore, Md. : 1950), 173 3, 1959-65Chabot, Sophie; Salez, Laurent; McCormack, Francis X; Touqui, Lhousseine; Chignard, Michel 2003. Surfactant protein A inhibits lipopolysaccharide-induced in vivo production of interleukin-10 by mononuclear phagocytes during lung inflammation. American journal of respiratory cell and molecular biology, 28 3, 347-53Head, James F; Mealy, Tanya R; McCormack, Francis X; Seaton, Barbara A 2003. Crystal structure of trimeric carbohydrate recognition and neck domains of surfactant protein A. The Journal of biological chemistry, 278 44, 43254-60Karbowniczek, Magdalena; Astrinidis, Aristotelis; Balsara, Binaifer R; Testa, Joseph R; Lium, James H; Colby, Thomas V; McCormack, Francis X; Henske, Elizabeth Petri 2003. Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism. American journal of respiratory and critical care medicine, 167 7, 976-82McCormack, Francis X; Gibbons, Reta; Ward, Susan R; Kuzmenko, Alexander; Wu, Huixing; Deepe, George S 2003. Macrophage-independent fungicidal action of the pulmonary collectins. The Journal of biological chemistry, 278 38, 36250-6Rodriguez Capote, Karina; McCormack, Francis X; Possmayer, Fred 2003. Pulmonary surfactant protein-A (SP-A) restores the surface properties of surfactant after oxidation by a mechanism that requires the Cys6 interchain disulfide bond and the phospholipid binding domain. The Journal of biological chemistry, 278 23, 20461-74Sietsema, Kathy E; McCormack, Francis X 2003. Lymphangioleiomyomatosis: insights about, and from, a rare disease. American journal of respiratory and critical care medicine, 168 12, 1405-6Wu, Huixing; Kuzmenko, Alexander; Wan, Sijue; Schaffer, Lyndsay; Weiss, Alison; Fisher, James H; Kim, Kwang Sik; McCormack, Francis X 2003. Surfactant proteins A and D inhibit the growth of Gram-negative bacteria by increasing membrane permeability. The Journal of clinical investigation, 111 10, 1589-602Beharka, Alison A; Gaynor, Cecilia D; Kang, Byoung K; Voelker, Dennis R; McCormack, Francis X; Schlesinger, Larry S 2002. Pulmonary surfactant protein A up-regulates activity of the mannose receptor, a pattern recognition receptor expressed on human macrophages. Journal of immunology (Baltimore, Md. : 1950), 169 7, 3565-73Guillot, Loïc; Balloy, Viviane; McCormack, Francis X; Golenbock, Douglas T; Chignard, Michel; Si-Tahar, Mustapha 2002. Cutting edge: the immunostimulatory activity of the lung surfactant protein-A involves Toll-like receptor 4. Journal of immunology (Baltimore, Md. : 1950), 168 12, 5989-92Malloy, Jaret L; Veldhuizen, Ruud A W; McCormack, Francis X; Korfhagen, Thomas R; Whitsett, Jeffery A; Lewis, James F 2002. Pulmonary surfactant and inflammation in septic adult mice: role of surfactant protein A. Journal of applied physiology (Bethesda, Md. : 1985), 92 2, 809-16McCormack, Francis X; Whitsett, Jeffrey A 2002. The pulmonary collectins, SP-A and SP-D, orchestrate innate immunity in the lung. The Journal of clinical investigation, 109 6, 707-12McCormack, Francis; Brody, Alan; Meyer, Christopher; Leonard, Jennifer; Chuck, Gail; Dabora, Sandra; Sethuraman, Gopalan; Colby, Thomas V; Kwiatkowski, David J; Franz, David N 2002. Pulmonary cysts consistent with lymphangioleiomyomatosis are common in women with tuberous sclerosis: genetic and radiographic analysis. Chest, 121 3 Suppl, 61SPalaniyar, Nades; Zhang, Liquian; Kuzmenko, Alexander; Ikegami, Machiko; Wan, Sijue; Wu, Huixing; Korfhagen, Thomas R; Whitsett, Jeffrey A; McCormack, Francis X 2002. The role of pulmonary collectin N-terminal domains in surfactant structure, function, and homeostasis in vivo. The Journal of biological chemistry, 277 30, 26971-9Franz, D N; Brody, A; Meyer, C; Leonard, J; Chuck, G; Dabora, S; Sethuraman, G; Colby, T V; Kwiatkowski, D J; McCormack, F X 2001. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. American journal of respiratory and critical care medicine, 164 4, 661-8Ikegami, M; Elhalwagi, B M; Palaniyar, N; Dienger, K; Korfhagen, T; Whitsett, J A; McCormack, F X 2001. The collagen-like region of surfactant protein A (SP-A) is required for correction of surfactant structural and functional defects in the SP-A null mouse. The Journal of biological chemistry, 276 42, 38542-8Linke, M J; Harris, C E; Korfhagen, T R; McCormack, F X; Ashbaugh, A D; Steele, P; Whitsett, J A; Walzer, P D 2001. Immunosuppressed surfactant protein A-deficient mice have increased susceptibility to Pneumocystis carinii infection. The Journal of infectious diseases, 183 6, 943-52Palaniyar, N; Ikegami, M; Korfhagen, T; Whitsett, J; McCormack, F X 2001. Domains of surfactant protein A that affect protein oligomerization, lipid structure and surface tension. Comparative biochemistry and physiology. Part A, Molecular & integrative physiology, 129 1, 109-27Salez, L; Balloy, V; van Rooijen, N; Lebastard, M; Touqui, L; McCormack, F X; Chignard, M 2001. Surfactant protein A suppresses lipopolysaccharide-induced IL-10 production by murine macrophages. Journal of immunology (Baltimore, Md. : 1950), 166 10, 6376-82Bridges, J P; Davis, H W; Damodarasamy, M; Kuroki, Y; Howles, G; Hui, D Y; McCormack, F X 2000. Pulmonary surfactant proteins A and D are potent endogenous inhibitors of lipid peroxidation and oxidative cellular injury. The Journal of biological chemistry, 275 49, 38848-55Damodarasamy, M; Zhang, M; Dienger, K; McCormack, F X 2000. Two rat surfactant protein A isoforms arise by a novel mechanism that includes alternative translation initiation. Biochemistry, 39 33, 10189-95Fisher, J H; Sheftelyevich, V; Ho, Y S; Fligiel, S; McCormack, F X; Korfhagen, T R; Whitsett, J A; Ikegami, M 2000. Pulmonary-specific expression of SP-D corrects pulmonary lipid accumulation in SP-D gene-targeted mice. American journal of physiology. Lung cellular and molecular physiology, 278 2, L365-73Palaniyar, N; McCormack, F X; Possmayer, F; Harauz, G 2000. Three-dimensional structure of rat surfactant protein A trimers in association with phospholipid monolayers. Biochemistry, 39 21, 6310-6Elhalwagi, B M; Zhang, M; Ikegami, M; Iwamoto, H S; Morris, R E; Miller, M L; Dienger, K; McCormack, F X 1999. Normal surfactant pool sizes and inhibition-resistant surfactant from mice that overexpress surfactant protein A. American journal of respiratory cell and molecular biology, 21 3, 380-7Ferguson, J S; Voelker, D R; McCormack, F X; Schlesinger, L S 1999. Surfactant protein D binds to Mycobacterium tuberculosis bacilli and lipoarabinomannan via carbohydrate-lectin interactions resulting in reduced phagocytosis of the bacteria by macrophages. Journal of immunology (Baltimore, Md. : 1950), 163 1, 312-21McCormack, F X; Damodarasamy, M; Elhalwagi, B M 1999. Deletion mapping of N-terminal domains of surfactant protein A. The N-terminal segment is required for phospholipid aggregation and specific inhibition of surfactant secretion. The Journal of biological chemistry, 274 5, 3173-81Yu, S H; McCormack, F X; Voelker, D R; Possmayer, F 1999. Interactions of pulmonary surfactant protein SP-A with monolayers of dipalmitoylphosphatidylcholine and cholesterol: roles of SP-A domains. Journal of lipid research, 40 5, 920-9Borron, P; McCormack, F X; Elhalwagi, B M; Chroneos, Z C; Lewis, J F; Zhu, S; Wright, J R; Shepherd, V L; Possmayer, F; Inchley, K; Fraher, L J 1998. Surfactant protein A inhibits T cell proliferation via its collagen-like tail and a 210-kDa receptor. The American journal of physiology, 275 4 Pt 1, L679-86McCormack, F X 1998. Structure, processing and properties of surfactant protein A. Biochimica et biophysica acta, 1408 2-3, 109-31Pattanajitvilai, S; Kuroki, Y; Tsunezawa, W; McCormack, F X; Voelker, D R 1998. Mutational analysis of Arg197 of rat surfactant protein A. His197 creates specific lipid uptake defects. The Journal of biological chemistry, 273 10, 5702-7Smolarek, T A; Wessner, L L; McCormack, F X; Mylet, J C; Menon, A G; Henske, E P 1998. Evidence that lymphangiomyomatosis is caused by TSC2 mutations: chromosome 16p13 loss of heterozygosity in angiomyolipomas and lymph nodes from women with lymphangiomyomatosis. American journal of human genetics, 62 4, 810-5Sunkin, S M; Linke, M J; McCormack, F X; Walzer, P D; Stringer, J R 1998. Identification of a putative precursor to the major surface glycoprotein of Pneumocystis carinii. Infection and immunity, 66 2, 741-6Tsunezawa, W; Sano, H; Sohma, H; McCormack, F X; Voelker, D R; Kuroki, Y 1998. Site-directed mutagenesis of surfactant protein A reveals dissociation of lipid aggregation and lipid uptake by alveolar type II cells. Biochimica et biophysica acta, 1387 1-2, 433-46Zhang, M; Damodarasamy, M; Elhalwagi, B M; McCormack, F X 1998. The longer isoform and Cys-1 disulfide bridge of rat surfactant protein A are not essential for phospholipid and type II cell interactions. Biochemistry, 37 47, 16481-8Blau, H; Riklis, S; Van Iwaarden, J F; McCormack, F X; Kalina, M 1997. Nitric oxide production by rat alveolar macrophages can be modulated in vitro by surfactant protein A. The American journal of physiology, 272 6 Pt 1, L1198-204Elhalwagi, B M; Damodarasamy, M; McCormack, F X 1997. Alternate amino terminal processing of surfactant protein A results in cysteinyl isoforms required for multimer formation. Biochemistry, 36 23, 7018-25McCormack, F 1997. The structure and function of surfactant protein-A. Chest, 111 6 Suppl, 114S-119SMcCormack, F X; Festa, A L; Andrews, R P; Linke, M; Walzer, P D 1997. The carbohydrate recognition domain of surfactant protein A mediates binding to the major surface glycoprotein of Pneumocystis carinii. Biochemistry, 36 26, 8092-9McCormack, F X; Pattanajitvilai, S; Stewart, J; Possmayer, F; Inchley, K; Voelker, D R 1997. The Cys6 intermolecular disulfide bond and the collagen-like region of rat SP-A play critical roles in interactions with alveolar type II cells and surfactant lipids. The Journal of biological chemistry, 272 44, 27971-9McCormack, F X; Stewart, J; Voelker, D R; Damodarasamy, M 1997. Alanine mutagenesis of surfactant protein A reveals that lipid binding and pH-dependent liposome aggregation are mediated by the carbohydrate recognition domain. Biochemistry, 36 45, 13963-71Gaynor, C D; McCormack, F X; Voelker, D R; McGowan, S E; Schlesinger, L S 1995. Pulmonary surfactant protein A mediates enhanced phagocytosis of Mycobacterium tuberculosis by a direct interaction with human macrophages. Journal of immunology (Baltimore, Md. : 1950), 155 11, 5343-51McCormack, F X; King, T E; Bucher, B L; Nielsen, L; Mason, R J; McCormac, F X 1995. Surfactant protein A predicts survival in idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine, 152 2, 751-9Kuroki, Y; McCormack, F X; Ogasawara, Y; Mason, R J; Voelker, D R 1994. Epitope mapping for monoclonal antibodies identifies functional domains of pulmonary surfactant protein A that interact with lipids. The Journal of biological chemistry, 269 47, 29793-800McCormack, F X; Calvert, H M; Watson, P A; Smith, D L; Mason, R J; Voelker, D R 1994. The structure and function of surfactant protein A. Hydroxyproline- and carbohydrate-deficient mutant proteins. The Journal of biological chemistry, 269 8, 5833-41McCormack, F X; Kuroki, Y; Stewart, J J; Mason, R J; Voelker, D R 1994. Surfactant protein A amino acids Glu195 and Arg197 are essential for receptor binding, phospholipid aggregation, regulation of secretion, and the facilitated uptake of phospholipid by type II cells. The Journal of biological chemistry, 269 47, 29801-7Ogasawara, Y; McCormack, F X; Mason, R J; Voelker, D R 1994. Chimeras of surfactant proteins A and D identify the carbohydrate recognition domains as essential for phospholipid interaction. The Journal of biological chemistry, 269 47, 29785-92de Carvalho, M S; McCormack, F X; Leslie, C C 1993. The 85-kDa, arachidonic acid-specific phospholipase A2 is expressed as an activated phosphoprotein in Sf9 cells. Archives of biochemistry and biophysics, 306 2, 534-40Kalina, M; McCormack, F X; Crowley, H; Voelker, D R; Mason, R J 1993. Internalization of surfactant protein A (SP-A) into lamellar bodies of rat alveolar type II cells in vitro. The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society, 41 1, 57-70Zimmerman, P E; Voelker, D R; McCormack, F X; Paulsrud, J R; Martin, W J 1992. 120-kD surface glycoprotein of Pneumocystis carinii is a ligand for surfactant protein A. The Journal of clinical investigation, 89 1, 143-9Fisher, J H; McCormack, F; Park, S S; Stelzner, T; Shannon, J M; Hofmann, T 1991. In vivo regulation of surfactant proteins by glucocorticoids. American journal of respiratory cell and molecular biology, 5 1, 63-70McCormack, F X; King, T E; Voelker, D R; Robinson, P C; Mason, R J 1991. Idiopathic pulmonary fibrosis. Abnormalities in the bronchoalveolar lavage content of surfactant protein A. The American review of respiratory disease, 144 1, 160-6McCormack, F X; Fisher, J H; Suwabe, A; Smith, D L; Shannon, J M; Voelker, D R 1990. Expression and characterization of rat surfactant protein A synthesized in Chinese hamster ovary cells. Biochimica et biophysica acta, 1087 2, 190-8Mackenzie, C F; Shin, B; Krishnaprasad, D; McCormack, F; Illingworth, W 1985. Assessment of cardiac and respiratory function during surgery on patients with acute quadriplegia. Journal of neurosurgery, 62 6, 843-9Sykes, J A; McCormack, F X; O'Brien, T J 1978. A preliminary study of the superoxide dismutase content of some human tumors. Cancer research, 38 9, 2759-62Kingma P.;Zhang L.;Ikegami M.;Hartshorn K.;McCormack F.;Whitsett J. 12-15-2006. Erratum: Correction of pulmonary abnormalities in Sftpd<sup>-/-</sup> mice requires the Journal of Biological Chemistry, 281 50, 38968Bridges J.P.;Davis H.W.;Damodarasamy M.;Kuroki Y.;Howles G.;Hui D.Y.;Francis-X. McCormack 12-13-2002. Erratum: Pulmonary surfactant proteins A and D are potent endogenous inhihitors of lipid peroxidatio Journal of Biological Chemistry, 277 50, 49090 White M.R.; Nikolaidis N.M.; McCormack F.; Crouch E.C.; Hartshorn K.L. 12-08-2021. Viral Evasion of Innate Immune Defense: The Case of Resistance of Pandemic H1N1 Influenza A Virus to Frontiers in Microbiology, 12 , Jose A.; Shah S.A.; Anwar N.; Jones C.R.; McCormack F.X.; Sherman K.E.; Elwing J.M. 12-01-2021. Predictors of outcomes following liver transplant in hepatopulmonary syndrome: An OPTN database anal Respiratory Medicine, 190 , Zhang S.;Chen Y.;Potvin E.;Sanschagrin F.;Levesque R.;McCormack F.;Lau G. 12-01-2005. Comparative signature-tagged mutagenesis identifies Pseudomonas factors conferring resistance to the PLoS Pathogens, 1 3, 0259-0268Blau H.;Riklis S.;Van Iwaarden J.;McCormack F.;Kalina M. 12-01-1997. Nitric oxide production by rat alveolar macrophages can be modulated in vitro by surfactant protein Pneumologie, 51 11, 1080Singla A.;Gupta N.;Apewokin S.;McCormack F. 11-02-2017. Sirolimus for the treatment of lymphangioleiomyomatosis Expert Opinion on Orphan Drugs, 5 11, 907-921 Northrup H.; Aronow M.E.; Bebin E.M.; Bissler J.; Darling T.N.; de Vries P.J.; Frost M.D.; Fuchs Z.; Gosnell E.S.; Gosnell E.S.; Gupta N.; Jansen A.C.; Jó?wiak S.; Kingswood J.C.; Knilans T.K.; McCormack F.X.; Pounders A.; Roberds S.L.; Rodriguez-Buritica D.F.; Rodriguez-Buritica D.F.; Roth J.; Sampson J.R.; Sparagana S.; Thiele E.A.; Weiner H.L.; Wheless J.W.; Towbin A.J.; Krueger D.A.; Annear N.M.P.; Bartels U.; Berhouma M.; Bissler J.J.; Budde K.; Byars A.; Chugani H.; Cowen E.W.; Crino P.B.; Curatolo P.; de Vries P.; Dilling D.F.; Dunn D.W.; Ekong R.; Ess K.C.; Franz D.N.; Frost M.; Fuchs Z.D.B.; Guay-Woodford L.; Haddad L.; Halbert A.; Hebert A.A.; Henske E.P.; Holmes G.L.; Hook D.; Hulbert J.; Jansen A.; Johnson S.R.; King B.; Kingswood J.C.; Koenig M.K.; Korf B.; Kwiatkowski D.J.; Moss J.; Mowat D.; Mowrey K.; Nabbout R.; Nellist M.D.; O'Callaghan F.; Patel U.; Roach E.S.; Romp R.; Rozenberg M.; Ruoss S.J.; Sahin M.; Sampson J.; Samuels J.A.; Sauter M.; Smith C.A.; Soltani K.; Srivastava S.; Stuart C.; Teng J.M.C.; Thiele E.A.; Trout A.; van Eeghen A.; Vanclooster S.; Wang H.Z.; Wataya-Kaneda M.; Witman P.; Wright T.; Wu J.Y.; Young L. 10-01-2021. Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Pediatric Neurology, 123 , 50-66 Zafar M.A.; Sengupta R.; Bates A.; Woods J.C.; Radchenko C.; McCormack F.X.; Panos R.J. 10-01-2021. Oral Positive Expiratory Pressure Device for Excessive Dynamic Airway Collapse Caused by Emphysema Chest, 160 4, e333-e337Kotloff R.;McCormack F. 09-01-2016. Rare Lung Diseases: Occasionally the “Horse” Has Stripes Clinics in Chest Medicine, 37 3, xv-xviMcCormack F.;Lee H.;Trapnell B. 07-21-2011. The authors reply New England Journal of Medicine, 365 3, 272McCormack F.;Henske E. 06-09-2010. Lymphangioleiomyomatosis and Pulmonary Disease in TSC Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics, , 345-368Bissler J.;Young L.;McCormack F. 05-01-2008. Dr. Bissler and colleagues reply New England Journal of Medicine, 358 18, 1964McCormack F.X.;Inoue Y.;Moss J.;Singer L.G.;Strange C.;Nakata K.;Barker A.F.;Chapman J.T.;Brantly M.L.;Stocks J.M.;Brown K.K.;Lynch J.P.;Goldberg H.J.;Young L.R.;Kinder B.W.;Downey G.P.;Sullivan E.J.;Colby T.V.;McKay R.T.;Cohen M.M.;Korbee L.;Taveira-DaSilva A.M.;Lee H.S.;Krischer J.P.;Trapnell B.C. 04-28-2011. Efficacy and safety of sirolimus in lymphangioleiomyomatosis New England Journal of Medicine, 364 17, 1595-1606McCormack F. 04-19-2016. Lymphangioleiomyomatosis Interstitial Pulmonary and Bronchiolar Disorders, , 747-768 Warrior K.; Leard L.E.; Nair A.R.; Gries C.J.; Fisher A.J.; Johnson S.R.; McCormack F.X.; Dilling D.F. 04-01-2022. A survey of use of mTOR inhibitors in patients with lymphangioleiomyomatosis listed for lung transpl Respiratory Medicine, 195 , Struik M.H.L.;Van Moorsel C.H.M.;Van Beek F.T.;Veltkamp M.;Mccormack F.X.;Grutters J.C. 04-01-2015. The importance of biobank and nationwide registry for lymphangioleiomyomatosis in a small sized coun Expert Opinion on Orphan Drugs, 3 4, 393-401Hagaman J.T.;Ferguson B.;Meyer C.;Wang J.;McCormack F.X.;Nath A. 04-01-2010. Endobronchial carcinoid tumor in an HIV-infected patient Journal of Bronchology and Interventional Pulmonology, 17 2, 174-176McCormack F. 03-31-2006. Author's Reply to "Reader's response to 'Lymphangioleiomyomatosis'" MedGenMed Medscape General Medicine, 8 1, Sontake V.; Wang Y.; Kasam R.K.; Sinner D.; Reddy G.B.; Naren A.P.; McCormack F.X.; White E.S.; Jegga A.G.; Madala S.K. 02-23-2017. Hsp90 regulation of fibroblast activation in pulmonary fibrosis JCI Insight, 2 4, Lee L.H.; Gasilina A.; Roychoudhury J.; Clark J.; McCormack F.X.; Pressey J.; Grimley M.S.; Lorsbach R.; Ali S.; Bailey M.; Stephens P.; Ross J.S.; Miller V.A.; Nassar N.N.; Kumar A.R. 02-09-2017. Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while i JCI Insight, 2 3, Hagaman J.;Panos R.;McCormack F.;Thakar C.;Wikenheiser-Brokamp K.;Shipley R.;Kinder B. 02-01-2011. Vitamin D deficiency and reduced lung function in connective tissue-associated interstitial lung dis Chest, 139 2, 353-360 Du Y.; Guo M.; Wu Y.; Wagner A.; Perl A.K.; Wikenheiser-Brokamp K.; Yu J.; Gupta N.; Kopras E.; Krymskaya V.; Obraztsova K.; Tang Y.; Kwiatkowski D.; Henske E.P.; McCormack F.; Xu Y. 01-01-2022. Lymphangioleiomyomatosis (LAM) Cell Atlas Thorax, , Cudzilo C.J.;Szczesniak R.D.;Brody A.S.;Rattan M.S.;Krueger D.A.;Bissler J.J.;Franz D.N.;McCormack F.X.;Young L.R. 01-01-2013. Lymphangioleiomyomatosis screening in women with tuberous sclerosis Chest, 144 2, 578-585Swigris J.;Lee H.;Cohen M.;Inoue Y.;Moss J.;Singer L.;Young L.;McCormack F. 01-01-2013. St. George's respiratory questionnaire has longitudinal construct validity in lymphangioleiomyomatos Chest, 143 6, 1671-1678Johnson S.R.;Cordier J.F.;Lazor R.;Cottin V.;Costabel U.;Harari S.;Reynaud-Gaubert M.;Boehler A.;Brauner M.;Popper H.;Bonetti F.;Kingswood C.;Albera C.;Bissler J.;Bouros D.;Corris P.;Donnelly S.;Durand C.;Egan J.;Grutters J.C.;Hodgson U.;Hollis G.;Korzeniewska-Kosela M.;Kus J.;Lacronique J.;Lammers J.W.;McCormack F.;Mendes A.C.;Moss J.;Naalsund A.;Pohl W.;Radzikowska E.;Robalo-Cordeiro C.;Rouvière O.;Ryu J.;Schiavina M.;Tattersfield A.E.;Travis W.;Tukiainen P.;Urban T.;Valeyre D.;Verleden G.M. 01-01-2010. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis European Respiratory Journal, 35 1, 14-26McCormack F. 01-01-2008. Response Chest, 134 1, 218 McCormack F.X. 01-01-2005. Other rare lung diseases: Lymphangioleiomyomatosis Respiratory Genetics, , 479-489 Young L.R.; McCormack F.X. 01-01-2005. Other rare lung diseases: Hermansky-Pudlak Syndrome Respiratory Genetics, , 491-498Almoosa K.F.;Khoury S.F.;McCormack F.X. 01-01-2003. Acute myocardial infarction complicating sudden interruption of chronic prostacyclin therapy for pul Clinical Pulmonary Medicine, 10 4, 231-234Malloy J.;Veldhuizen R.;McCormack F.;Korfhagen T.;Whitsett J.;Lewis J. 01-01-2002. Pulmonary surfactant and inflammation in septic adult mice: Role of surfactant protein A Journal of Applied Physiology, 92 2, 809-816McCormack F.;King T.;Bucher B.;Nielsen L.;Mason R. 01-01-1995. Erratum: Surfactant protein A predicts survival in idiopathic pulmonary fibrosis (American Journal o American Journal of Respiratory and Critical Care Medicine, 152 4 I, 1425McCormack F.X. 01-01-1995. Molecular biology of the surfactant apoproteins Seminars in Respiratory and Critical Care Medicine, 16 1, 29-38De Carvalho M.;McCormack F.;Leslie C. 01-01-1993. The 85-kDa, Arachidonic Acid-Specific Phospholipase A<inf>2</inf> Is Expressed as an Act Archives of Biochemistry and Biophysics, 306 2, 534-540McCormack, F X . Functional mapping of surfactant protein A. Pediatric pathology & molecular medicine, 20 4, 293-318mccormack, f.x. . , , McCormack, Francis X . The way forward in lymphangioleiomyomatosis: a trial for every patient, every patient in a trial. Jornal brasileiro de pneumologia : publicaça?o oficial da Sociedade Brasileira de Pneumologia e Tisilogia, 37 4, 422-3
Research InterestsBasic laboratory focus 1. The structure/function relationships of surfactant proteins, surfactant homeostasis, surfactant proteins (collectins) in host defense. 2. Mechanisms of pulmonary fibrosis 3. The role of the pulmonary epithelium in influenza infection 4. Molecular and cellular basis of pulmonary alveolar microlithiasis 5. Pathogenesis of pulmonary Langerhans histiocytosis Clinical research focus 1. Genetic interstitial lung disea...
Clinical Interests Critical Care MedicineLung DiseasePulmonary DiseaseRare Lung DiseaseSarcoidosisAsbestosisBronchiolitisChronic CoughDyspneaHistoplasmosisIdiopathic Pulmonary FibrosisLegionnaires' Disease (Legionellosis)Lymphangioleiomyomatosis (LAM)Lymphatic DiseasePleurisyPneumoniaPneumonitisPulmonary FibrosisPulmonary SarcoidosisRespiration DisordersRespiratory Syncytial Virus (RSV)Respiratory Tract DiseaseRespiratory Tract InfectionsShortness of BreathThoracic DiseaseTuberculosisWhooping Cough (Pertussis)
Peer Reviewed Publications Gibbons, Erin; Taya, Manisha; Wu, Huixing; Lopa, Samia H; Moss, Joel; Henske, Elizabeth P; McCormack, Francis X; Hammes, Stephen R 2024. Glycoprotein non-metastatic melanoma protein B promotes tumor growth and is a biomarker for lymphangioleiomyomatosis. Endocrine-related cancer, 31 6, Gomez-Arroyo, Jose; Houweling, Arjan C; Bogaard, Harm Jan; Aman, Jurjan; Kitzmiller, Joseph A; Porollo, Aleksey; Dooijes, Dennis; Meijboom, Lilian J; Hale, Phillip; Pauciulo, Michael W; Hong, Jason; Zhu, Na; Welch, Carrie; Shen, Yufeng; Zacharias, William J; McCormack, Francis X; Aldred, Micheala A; Weirauch, Matthew T; Graf, Stefan; Rhodes, Christopher; Chung, Wendy K; Whitsett, Jeffrey A; Martin, Lisa J; Kalinichenko, Vladimir V; Nichols, William C 2024. Role of Forkhead box F1 in the Pathobiology of Pulmonary Arterial Hypertension. bioRxiv : the preprint server for biology, , Hasegawa, Yoshihiro; Franks, Jennifer M; Tanaka, Yusuke; Uehara, Yasuaki; Read, David F; Williams, Claire; Srivatsan, Sanjay; Pitstick, Lori B; Nikolaidis, Nikolaos M; Shaver, Ciara M; Kropski, Jonathan; Ware, Lorraine B; Taylor, Chase J; Banovich, Nicholas E; Wu, Huixing; Gardner, Jason C; Osterburg, Andrew R; Yu, Jane J; Kopras, Elizabeth J; Teitelbaum, Steven L; Wikenheiser-Brokamp, Kathryn A; Trapnell, Cole; McCormack, Francis X 2024. Pulmonary osteoclast-like cells in silica induced pulmonary fibrosis. Science advances, 10 28, eadl4913Jose, Arun; Rahman, Natalia; Opotowsky, Alexander R; Glorioso, Thomas J; Waldo, Stephen W; Zeder, Katarina; Seto, Arnold; Elwing, Jean M; McCormack, Francis X; Maron, Bradley A 2024. Association of Cardiopulmonary Hemodynamics and Mortality in Veterans With Liver Cirrhosis: A Retrospective Cohort Study. Journal of the American Heart Association, 13 8, e033847Rubin, Rhea; Baldi, Bruno Guedes; Shaw, Brian M; Kingsberg, Sheryl; Kopras, Elizabeth; Larkin, Lisa; McCormack, Francis X; Gupta, Nishant 2024. Hemoptysis Associated with Sexual Activity in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Akkenepally, Santhoshi V; Yombo, Dan J K; Yerubandi, Sanjana; Reddy, Geereddy Bhanuprakash; Deshpande, Deepak A; McCormack, Francis X; Madala, Satish K 2023. Interleukin 31 receptor α promotes smooth muscle cell contraction and airway hyperresponsiveness in asthma. Nature communications, 14 1, 8207Astrinidis, Aristotelis; Li, Chenggang; Zhang, Erik Y; Zhao, Xueheng; Zhao, Shuyang; Guo, Minzhe; Olatoke, Tasnim; Mattam, Ushodaya; Huang, Rong; Zhang, Alan G; Pitstick, Lori; Kopras, Elizabeth J; Gupta, Nishant; Jandarov, Roman; Smith, Eric P; Fugate, Elizabeth; Lindquist, Diana; Markiewski, Maciej M; Karbowniczek, Magdalena; Wikenheiser-Brokamp, Kathryn A; Setchell, Kenneth D R; McCormack, Francis X; Xu, Yan; Yu, Jane J 2023. Upregulation of acid ceramidase contributes to tumor progression in tuberous sclerosis complex. JCI insight, 8 9, Du, Yina; Guo, Minzhe; Wu, Yixin; Wagner, Andrew; Perl, Anne Karina; Wikenheiser-Brokamp, Kathryn; Yu, Jane; Gupta, Nishant; Kopras, Elizabeth; Krymskaya, Vera; Obraztsova, Kseniya; Tang, Yan; Kwiatkowski, David; Henske, Elizabeth P; McCormack, Francis; Xu, Yan 2023. Lymphangioleiomyomatosis (LAM) Cell Atlas. Thorax, 78 1, 85-87Evans, Jilly F; McCormack, Francis X; Sonenberg, Nahum; Krymskaya, Vera P 2023. Lost in translation: a neglected mTOR target for lymphangioleiomyomatosis. European respiratory review : an official journal of the European Respiratory Society, 32 169, Gajjala, Prathibha R; Singh, Priyanka; Odayar, Varshini; Ediga, Harshavardhana H; McCormack, Francis X; Madala, Satish K 2023. Wilms Tumor 1-Driven Fibroblast Activation and Subpleural Thickening in Idiopathic Pulmonary Fibrosis. International journal of molecular sciences, 24 3, Gupta, Nishant; Zhang, Bin; Zhou, Yuan; McCormack, Francis X; Ingledue, Rebecca; Robbins, Nathan; Kopras, Elizabeth J; McMahan, Susan; Singla, Abhishek; Swigris, Jeffrey; Cole, Adam G; Holz, Marina K 2023. Safety and Efficacy of Combined Resveratrol and Sirolimus in Lymphangioleiomyomatosis. Chest, 163 5, 1144-1155Harun, Nusrat; Gupta, Nishant; McCormack, Francis X; Macaluso, Maurizio 2023. Dynamic use of historical controls in clinical trials for rare disease research: A re-evaluation of the MILES trial. Clinical trials (London, England), 20 3, 223-234Hasegawa, Yoshihiro; Franks, Jennifer M; Tanaka, Yusuke; Uehara, Yasuaki; Read, David F; Williams, Claire; Srivatsan, Sanjay; Pitstick, Lori B; Nikolaidis, Nikolaos M; Shaver, Ciara M; Wu, Huixing; Gardner, Jason C; Osterburg, Andrew R; Yu, Jane J; Kopras, Elizabeth J; Teitelbaum, Steven L; Wikenheiser-Brokamp, Kathryn A; Trapnell, Cole; McCormack, Francis X 2023. Pulmonary osteoclast-like cells in silica induced pulmonary fibrosis. bioRxiv : the preprint server for biology, , Jose, Arun; Elwing, Jean M; Kawut, Steven M; Pauciulo, Michael W; Sherman, Kenneth E; Nichols, William C; Fallon, Michael B; McCormack, Francis X 2023. Human liver single nuclear RNA sequencing implicates BMPR2, GDF15, arginine, and estrogen in portopulmonary hypertension. Communications biology, 6 1, 826Lin, Hong T; Wikenheiser-Brokamp, Kathryn A; Udstuen, Gavin; Jones, Blaise; McCormack, Francis X 2023. Marked Improvement in Soft Tissue and CNS Manifestations of Adult Langerhans Cell Histiocytosis on Targeted MEK Inhibitor Therapy. Chest, 163 2, e53-e56McCormack, Fiona C; Hopley, Rachel C; Boath, Elizabeth H; Parry, Sian L; Roscoe, Suzie M; Stewart, Antony; Birch, Victoria A 2023. Exploring pregnant women's experiences of stopping smoking with an incentive scheme with 'enhanced' support: a qualitative study. Perspectives in public health, 143 5, 285-291Olatoke, Tasnim; Wagner, Andrew; Astrinidis, Aristotelis; Zhang, Erik Y; Guo, Minzhe; Zhang, Alan G; Mattam, Ushodaya; Kopras, Elizabeth J; Gupta, Nishant; Smith, Eric P; Karbowniczek, Magdalena; Markiewski, Maciej M; Wikenheiser-Brokamp, Kathryn A; Whitsett, Jeffrey A; McCormack, Francis X; Xu, Yan; Yu, Jane J 2023. Single-cell multiomic analysis identifies a HOX-PBX gene network regulating the survival of lymphangioleiomyomatosis cells. Science advances, 9 19, eadf8549Uehara, Yasuaki; Tanaka, Yusuke; Zhao, Shuyang; Nikolaidis, Nikolaos M; Pitstick, Lori B; Wu, Huixing; Yu, Jane J; Zhang, Erik; Hasegawa, Yoshihiro; Noel, John G; Gardner, Jason C; Kopras, Elizabeth J; Haffey, Wendy D; Greis, Kenneth D; Guo, Jinbang; Woods, Jason C; Wikenheiser-Brokamp, Kathryn A; Kyle, Jennifer E; Ansong, Charles; Teitelbaum, Steven L; Inoue, Yoshikazu; Altinişik, Göksel; Xu, Yan; McCormack, Francis X 2023. Insights into pulmonary phosphate homeostasis and osteoclastogenesis emerge from the study of pulmonary alveolar microlithiasis. Nature communications, 14 1, 1205Chernbumroong, Saisakul; Johnson, Janice; Gupta, Nishant; Miller, Suzanne; McCormack, Francis X; Garibaldi, Jonathan M; Johnson, Simon R 2021. Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis. The European respiratory journal, 57 6, Foley, M; Duffy, F; Skally, M; McCormack, F; Finn, C; O'Connor, M; Cafferkey, J; Thomas, T; Burns, K; Fitzpatrick, F; O'Connell, K; Smyth, E G; Humphreys, H 2021. Evolving epidemiology of carbapenemase-producing Enterobacterales: one hospital's infection prevention and control response over nine years. The Journal of hospital infection, 112 , 61-68McCarthy, Cormac; Gupta, Nishant; Johnson, Simon R; Yu, Jane J; McCormack, Francis X 2021. Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. The Lancet. Respiratory medicine, , Northrup, Hope; Aronow, Mary E; Bebin, E Martina; Bissler, John; Darling, Thomas N; de Vries, Petrus J; Frost, Michael D; Fuchs, Zoë; Gosnell, Elizabeth S; Gupta, Nishant; Jansen, Anna C; Jó?wiak, Sergiusz; Kingswood, J Chris; Knilans, Timothy K; McCormack, Francis X; Pounders, Ashley; Roberds, Steven L; Rodriguez-Buritica, David F; Roth, Jonathan; Sampson, Julian R; Sparagana, Steven; Thiele, Elizabeth Anne; Weiner, Howard L; Wheless, James W; Towbin, Alexander J; Krueger, Darcy A 2021. Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations. Pediatric neurology, 123 , 50-66Tian, Xinlun; Glass, Jennifer E; Kwiatkowski, David J; Towbin, Alexander J; Li, Yinan; Sund, Kristen L; Krueger, Darcy A; Franz, David N; McCormack, Francis X; Gupta, Nishant 2021. Lymphangioleiomyomatosis Association with Underlying Genotype in Patients with Tuberous Sclerosis Complex. Annals of the American Thoracic Society, 18 5, 815-819White, Mitchell R; Nikolaidis, Nikolaos M; McCormack, Francis; Crouch, Erika C; Hartshorn, Kevan L 2021. Viral Evasion of Innate Immune Defense: The Case of Resistance of Pandemic H1N1 Influenza A Virus to Human Mannose-Binding Proteins. Frontiers in microbiology, 12 , 774711Dellinger, Michael T; McCormack, Francis X 2020. The emergence of targetable MEKanisms in sporadic lymphatic disorders. EMBO molecular medicine, , e12822Guo, Minzhe; Yu, Jane J; Perl, Anne Karina; Wikenheiser-Brokamp, Kathryn A; Riccetti, Matt; Zhang, Erik Y; Sudha, Parvathi; Adam, Mike; Potter, Andrew; Kopras, Elizabeth J; Giannikou, Krinio; Potter, S Steven; Sherman, Sue; Hammes, Stephen R; Kwiatkowski, David J; Whitsett, Jeffrey A; McCormack, Francis X; Xu, Yan 2020. Single Cell Transcriptomic Analysis Identifies a Unique Pulmonary Lymphangioleiomyomatosis Cell. American journal of respiratory and critical care medicine, , Gupta, Nishant; Bleesing, Jack H; McCormack, Francis X 2020. Successful Response to Treatment with Sirolimus in Pulmonary Sarcoidosis. American journal of respiratory and critical care medicine, , Gupta, Nishant; Hagner, Miles; Wu, Huixing; Young, Lisa R; Palipana, Anushka; Szczesniak, Rhonda D; McCormack, Francis X 2020. Serum Vascular Endothelial Growth Factor-C as a Marker of Therapeutic Response to Sirolimus in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Jönsson, Åsa Lina M; Bendstrup, Elisabeth; Mogensen, Susie; Kopras, Elizabeth J; McCormack, Francis X; Campo, Ilaria; Mariani, Francesca; Escribano-Montaner, Amparo; Holm, Are M; Martinez-Colls, Maria Del Mar; Pintos-Morell, Guillem; Taillé, Camille; Crestani, Bruno; Hilberg, Ole; Hvarregaard Christensen, Jane; Simonsen, Ulf 2020. Eight novel variants in the SLC34A2 gene in pulmonary alveolar microlithiasis. The European respiratory journal, 55 2, Kosciuk, Patrick; Meyer, Cristopher; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2020. Pulmonary alveolar microlithiasis. European respiratory review : an official journal of the European Respiratory Society, 29 158, Krymskaya, Vera P; Courtwright, Andrew M; Fleck, Victoria; Dorgan, Daniel; Kotloff, Robert; McCormack, Francis X; Kreider, Maryl 2020. A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex. Respiratory medicine, 163 , 105898Liu, Huan; Osterburg, Andrew R; Flury, Jennifer; Swank, Zulma; McGraw, Dennis W; Gupta, Nishant; Wikenheiser-Brokamp, Kathryn A; Kumar, Ashish; Tazi, Abdellatif; Inoue, Yoshikazu; Hirose, Masaki; McCormack, Francis X; Borchers, Michael T 2020. MAPK mutations and cigarette smoke promote the pathogenesis of pulmonary Langerhans cell histiocytosis. JCI insight, 5 4, Poovelikunnel, Toney T; Duffy, Fionnuala; Puthussery, Thressia; Gangadharan, Shaini; McCormack, Fiona; Carpenter, Helen; Kizhakedath, Mary; Hawkshaw, Susan 2020. Clinically indicated replacement of peripheral vascular catheters: is it safe for patients? British journal of nursing (Mark Allen Publishing), 29 8, S4-S10Shaw, Brian M; Shaw, Steven D; McCormack, Francis X 2020. Pulmonary Alveolar Microlithiasis. Seminars in respiratory and critical care medicine, 41 2, 280-287Gupta, Nishant; Lee, Hye-Seung; Ryu, Jay H; Taveira-DaSilva, Angelo M; Beck, Gerald J; Lee, Jar-Chi; McCarthy, Kevin; Finlay, Geraldine A; Brown, Kevin K; Ruoss, Stephen J; Avila, Nilo A; Moss, Joel; McCormack, Francis X 2019. The NHLBI LAM Registry: Prognostic Physiologic and Radiologic Biomarkers Emerge From a 15-Year Prospective Longitudinal Analysis. Chest, 155 2, 288-296Gupta, Nishant; Lee, Hye-Seung; Young, Lisa R; Strange, Charlie; Moss, Joel; Singer, Lianne G; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Downey, Gregory P; Taveira-DaSilva, Angelo M; Krischer, Jeffrey P; Setchell, Kenneth; Trapnell, Bruce C; Inoue, Yoshikazu; McCormack, Francis X 2019. Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis. The European respiratory journal, 53 4, Hirose, Masaki; Matsumuro, Akiko; Arai, Toru; Sugimoto, Chikatoshi; Akira, Masanori; Kitaichi, Masanori; Young, Lisa R; McCormack, Francis X; Inoue, Yoshikazu 2019. Serum vascular endothelial growth factor-D as a diagnostic and therapeutic biomarker for lymphangioleiomyomatosis. PloS one, 14 2, e0212776McCarthy, Cormac; Bartholmai, Brian J; Woods, Jason C; McCormack, Francis X; Trapnell, Bruce C 2019. Automated Parenchymal Pattern Analysis of Treatment Responses in Pulmonary Alveolar Proteinosis. American journal of respiratory and critical care medicine, , McCarthy, Cormac; McCormack, Francis X 2019. Recurrent Sinusitis, Lower Limb Edema, and Nail Changes. Annals of the American Thoracic Society, 16 6, 752-755Nalian, Armen; Umstead, Todd M; Yang, Ching-Hui; Silveyra, Patricia; Thomas, Neal J; Floros, Joanna; McCormack, Francis X; Chroneos, Zissis C 2019. Structural and Functional Determinants of Rodent and Human Surfactant Protein A: A Synthesis of Binding and Computational Data. Frontiers in immunology, 10 , 2613Walkup, Laura L; Roach, David J; Hall, Chase S; Gupta, Nishant; Thomen, Robert P; Cleveland, Zackary I; McCormack, Francis X; Woods, Jason C 2019. Cyst Ventilation Heterogeneity and Alveolar Airspace Dilation as Early Disease Markers in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 16 8, 1008-1016Zak, Sara; Mokhallati, Nadine; Su, Weiji; McCormack, Francis X; Franz, David N; Mays, Maxwell; Krueger, Darcy A; Szczesniak, Rhonda D; Gupta, Nishant 2019. Lymphangioleiomyomatosis Mortality in Patients with Tuberous Sclerosis Complex. Annals of the American Thoracic Society, 16 4, 509-512Gupta, Nishant; Colby, Thomas V; Meyer, Cristopher A; McCormack, Francis X; Wikenheiser-Brokamp, Kathryn A 2018. Smoking-Related Diffuse Cystic Lung Disease. Chest, 154 2, e31-e35Gupta, Nishant; Johnson, Simon R; Moss, Joel; McCormack, Francis X 2018. Reply to Yanagisawa: Treatment of Pulmonary Lymphangioleiomyomatosis during Pregnancy. American journal of respiratory and critical care medicine, 197 11, 1507-1508Liu, Huan; Osterburg, Andrew R; Flury, Jennifer; Huang, Shuo; McCormack, Francis X; Cormier, Stephania A; Borchers, Michael T 2018. NKG2D Regulation of Lung Pathology and Dendritic Cell Function Following Respiratory Syncytial Virus Infection. The Journal of infectious diseases, 218 11, 1822-1832Miller, Suzanne; Coveney, Clare; Johnson, Janice; Farmaki, Aliki-Eleni; Gupta, Nishant; Tobin, Martin D; Wain, Louise V; McCormack, Francis X; Boocock, David J; Johnson, Simon R 2018. The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis. The European respiratory journal, 52 5, Feemster, Laura C; Lyons, Patrick G; Chatterjee, Rohini S; Kidambi, Pranav; McCormack, Francis X; Moss, Joel; Wilson, Kevin C; Thomson, Carey C 2017. Summary for Clinicians: Lymphangioleiomyomatosis Diagnosis and Management Clinical Practice Guideline. Annals of the American Thoracic Society, 14 7, 1073-1075Gupta, Nishant; Finlay, Geraldine A; Kotloff, Robert M; Strange, Charlie; Wilson, Kevin C; Young, Lisa R; Taveira-DaSilva, Angelo M; Johnson, Simon R; Cottin, Vincent; Sahn, Steven A; Ryu, Jay H; Seyama, Kuniaki; Inoue, Yoshikazu; Downey, Gregory P; Han, MeiLan K; Colby, Thomas V; Wikenheiser-Brokamp, Kathryn A; Meyer, Cristopher A; Smith, Karen; Moss, Joel; McCormack, Francis X 2017. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. American journal of respiratory and critical care medicine, 196 10, 1337-1348Gupta, Nishant; Kopras, Elizabeth J; Henske, Elizabeth P; James, Laura E; El-Chemaly, Souheil; Veeraraghavan, Srihari; Drake, Matthew G; McCormack, Francis X 2017. Spontaneous Pneumothoraces in Patients with Birt-Hogg-Dubé Syndrome. Annals of the American Thoracic Society, 14 5, 706-713Gupta, Nishant; Langenderfer, Dale; McCormack, Francis X; Schauer, Daniel P; Eckman, Mark H 2017. Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective. Annals of the American Thoracic Society, 14 1, 17-25Han, MeiLan K; Tyburczy, Magdalena E; Darling, Thomas N; Kazerooni, Ella A; Myers, Jeffrey L; McCormack, Francis X; Moss, Joel; Kwiatkowski, David J 2017. Apparent Sporadic Lymphangioleiomyomatosis (LAM) in a Man Due to Extreme Mosaicism for a TSC2 Mutation. Annals of the American Thoracic Society, , Kristof, Arnold S; Petrof, Basil J; Hamid, Qutayba; Kolb, Martin; Landry, Jennifer S; MacKenzie, Alex; McCormack, Francis X; Murawski, Inga J; Moss, Joel; Rauch, Frank; Rosas, Ivan O; Shapiro, Adam J; Smith, Benjamin M; Thomas, David Y; Trapnell, Bruce C; Young, Lisa R; Zariwala, Maimoona A 2017. An Official American Thoracic Society Workshop Report: Translational Research in Rare Respiratory Diseases. Annals of the American Thoracic Society, 14 8, 1239-1247Krymskaya, Vera P; McCormack, Francis X 2017. Lymphangioleiomyomatosis: A Monogenic Model of Malignancy. Annual review of medicine, 68 , 69-83Lee, Lynn H; Gasilina, Anjelika; Roychoudhury, Jayeeta; Clark, Jason; McCormack, Francis X; Pressey, Joseph; Grimley, Michael S; Lorsbach, Robert; Ali, Siraj; Bailey, Mark; Stephens, Philip; Ross, Jeffrey S; Miller, Vincent A; Nassar, Nicolas N; Kumar, Ashish R 2017. Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes. JCI insight, 2 3, e89473Liu, Huan; Jakubzick, Claudia; Osterburg, Andrew R; Nelson, Rebecca L; Gupta, Nishant; McCormack, Francis X; Borchers, Michael T 2017. Dendritic Cell Trafficking and Function in Rare Lung Diseases. American journal of respiratory cell and molecular biology, , Lu, Calvin; Lee, Hye-Seung; Pappas, George P; Dilling, Daniel F; Burger, Charles D; Shifren, Adrian; Veeraraghavan, Srihari; Chapman, Jeffrey T; Parambil, Joseph; Ruoss, Stephen J; Young, Lisa R; Hammes, Stephen R; Kopras, Elizabeth J; Roads, Tammy; Krischer, Jeffrey P; McCormack, Francis X 2017. A Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 14 6, 919-928McCarthy, Cormac; Lara Gallego, Beatriz; Trapnell, Bruce C; McCormack, Francis X 2017. Epidemiology of Rare Lung Diseases: The Challenges and Opportunities to Improve Research and Knowledge. Advances in experimental medicine and biology, 1031 , 419-442McCormack, Francis X; Gupta, Nishant; Finlay, Geraldine; Young, Lisa R; Johnson, Simon; Cottin, Vincent; Wilson, Kevin; Moss, Joel 2017. Reply to Official ATS/JRS Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. American journal of respiratory and critical care medicine, , Nikolaidis, Nikolaos M; Noel, John G; Pitstick, Lori B; Gardner, Jason C; Uehara, Yasuaki; Wu, Huixing; Saito, Atsushi; Lewnard, Kara E; Liu, Huan; White, Mitchell R; Hartshorn, Kevan L; McCormack, Francis X 2017. Mitogenic stimulation accelerates influenza-induced mortality by increasing susceptibility of alveolar type II cells to infection. Proceedings of the National Academy of Sciences of the United States of America, 114 32, E6613-E6622Noel, John G; Ramser, Benjamin J; Cancelas, Jose A; McCormack, Francis X; Gardner, Jason C 2017. Thermal injury of the skin induces G-CSF-dependent attenuation of EPO-mediated STAT signaling and erythroid differentiation arrest in mice. Experimental hematology, , Rynkiewicz, Michael J; Wu, Huixing; Cafarella, Tanya R; Nikolaidis, Nikolaos M; Head, James F; Seaton, Barbara A; McCormack, Francis X 2017. Differential Ligand Binding Specificities of the Pulmonary Collectins Are Determined by the Conformational Freedom of a Surface Loop. Biochemistry, 56 31, 4095-4105Sontake, Vishwaraj; Wang, Yunguan; Kasam, Rajesh K; Sinner, Debora; Reddy, Geereddy B; Naren, Anjaparavanda P; McCormack, Francis X; White, Eric S; Jegga, Anil G; Madala, Satish K 2017. Hsp90 regulation of fibroblast activation in pulmonary fibrosis. JCI insight, 2 4, e91454Wang, Yunguan; Yella, Jaswanth; Chen, Jing; McCormack, Francis X; Madala, Satish K; Jegga, Anil G 2017. Unsupervised gene expression analyses identify IPF-severity correlated signatures, associated genes and biomarkers. BMC pulmonary medicine, 17 1, 133Argula, Rahul G; Kokosi, Maria; Lo, Pechin; Kim, Hyun J; Ravenel, James G; Meyer, Cristopher; Goldin, Jonathan; Lee, Hye-Seung; Strange, Charlie; McCormack, Francis X 2016. A Novel Quantitative Computed Tomographic Analysis Suggests How Sirolimus Stabilizes Progressive Air Trapping in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 13 3, 342-9Gardner, Jason C; Wu, Huixing; Noel, John G; Ramser, Benjamin J; Pitstick, Lori; Saito, Atsushi; Nikolaidis, Nikolaos M; McCormack, Francis X 2016. Keratinocyte growth factor supports pulmonary innate immune defense through maintenance of alveolar antimicrobial protein levels and macrophage function. American journal of physiology. Lung cellular and molecular physiology, 310 9, L868-79Goh, Boon Chong; Wu, Huixing; Rynkiewicz, Michael J; Schulten, Klaus; Seaton, Barbara A; McCormack, Francis X 2016. Elucidation of Lipid Binding Sites on Lung Surfactant Protein A Using X-ray Crystallography, Mutagenesis, and Molecular Dynamics Simulations. Biochemistry, 55 26, 3692-701Gupta, Nishant; Wikenheiser-Brokamp, Kathryn A; Fischer, Aryeh; McCormack, Francis X 2016. Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren Syndrome. Annals of the American Thoracic Society, 13 3, 371-5Itkin, Maxim G; McCormack, Francis X; Dori, Yoav 2016. Diagnosis and Treatment of Lymphatic Plastic Bronchitis in Adults Using Advanced Lymphatic Imaging and Percutaneous Embolization. Annals of the American Thoracic Society, 13 10, 1689-1696Itkin, Maxim; McCormack, Francis X 2016. Nonmalignant Adult Thoracic Lymphatic Disorders. Clinics in chest medicine, 37 3, 409-20Kotloff, Robert M; McCormack, Francis X 2016. Rare Lung Diseases: Occasionally the "Horse" Has Stripes. Clinics in chest medicine, 37 3, xv-xviMcCormack, Francis X; Gupta, Nishant; Finlay, Geraldine R; Young, Lisa R; Taveira-DaSilva, Angelo M; Glasgow, Connie G; Steagall, Wendy K; Johnson, Simon R; Sahn, Steven A; Ryu, Jay H; Strange, Charlie; Seyama, Kuniaki; Sullivan, Eugene J; Kotloff, Robert M; Downey, Gregory P; Chapman, Jeffrey T; Han, MeiLan K; D'Armiento, Jeanine M; Inoue, Yoshikazu; Henske, Elizabeth P; Bissler, John J; Colby, Thomas V; Kinder, Brent W; Wikenheiser-Brokamp, Kathryn A; Brown, Kevin K; Cordier, Jean F; Meyer, Cristopher; Cottin, Vincent; Brozek, Jan L; Smith, Karen; Wilson, Kevin C; Moss, Joel 2016. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. American journal of respiratory and critical care medicine, 194 6, 748-61Osterburg, Andrew R; Nelson, Rebecca L; Yaniv, Benyamin Z; Foot, Rachel; Donica, Walter R F; Nashu, Madison A; Liu, Huan; Wikenheiser-Brokamp, Kathryn A; Moss, Joel; Gupta, Nishant; McCormack, Francis X; Borchers, Michael T 2016. NK cell activating receptor ligand expression in lymphangioleiomyomatosis is associated with lung function decline. JCI insight, 1 16, e87270Saito, Atsushi; McCormack, Francis X 2016. Pulmonary Alveolar Microlithiasis. Clinics in chest medicine, 37 3, 441-8Soleimani, Manoocher; Barone, Sharon; Xu, Jie; Alshahrani, Saeed; Brooks, Marybeth; McCormack, Francis X; Smith, Roger D; Zahedi, Kamyar 2016. Prostaglandin-E2 Mediated Increase in Calcium and Phosphate Excretion in a Mouse Model of Distal Nephron Salt Wasting. PloS one, 11 7, e0159804Takada, Toshinori; Mikami, Ayako; Kitamura, Nobutaka; Seyama, Kuniaki; Inoue, Yoshikazu; Nagai, Katsura; Suzuki, Masaru; Moriyama, Hiroshi; Akasaka, Keiichi; Tazawa, Ryushi; Hirai, Toyohiro; Mishima, Michiaki; Hayashida, Mie; Hirose, Masaki; Sugimoto, Chikatoshi; Arai, Toru; Hattori, Noboru; Watanabe, Kentaro; Tamada, Tsutomu; Yoshizawa, Hirohisa; Akazawa, Kohei; Tanaka, Takahiro; Yagi, Keita; Young, Lisa R; McCormack, Francis X; Nakata, Koh 2016. Efficacy and Safety of Long-term Sirolimus Therapy for Asian Patients with Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Goldberg, Hilary J; Harari, Sergio; Cottin, Vincent; Rosas, Ivan O; Peters, Elizabeth; Biswal, Shibadas; Cheng, Yi; Khindri, Sanjeev; Kovarik, John M; Ma, Shenglin; McCormack, Francis X; Henske, Elizabeth Petri 2015. Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study. The European respiratory journal, 46 3, 783-94Gupta, Nishant; Han, MeiLan K; McCormack, Francis X 2015. Regional Sparing in an Oligemic Lung Segment Supports Hematogenous Spread as a Pathogenic Mechanism in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 12 8, 1247-8Gupta, Nishant; Meraj, Riffat; Tanase, Daniel; James, Laura E; Seyama, Kuniaki; Lynch, David A; Akira, Masanori; Meyer, Cristopher A; Ruoss, Stephen J; Burger, Charles D; Young, Lisa R; Almoosa, Khalid F; Veeraraghavan, Srihari; Barker, Alan F; Lee, Augustine S; Dilling, Daniel F; Inoue, Yoshikazu; Cudzilo, Corey J; Zafar, Muhammad A; McCormack, Francis X 2015. Accuracy of chest high-resolution computed tomography in diagnosing diffuse cystic lung diseases. The European respiratory journal, 46 4, 1196-9Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2015. Diffuse Cystic Lung Disease. Part II. American journal of respiratory and critical care medicine, 192 1, 17-29Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2015. Diffuse Cystic Lung Disease. Part I. American journal of respiratory and critical care medicine, 191 12, 1354-66Gupta, Rajeev; McCormack, Francis X 2015. Response. Lymphology, 48 1, 52Mulhall, Aaron M; McCormack, Francis X 2015. A 25-year-old man with fatigue, dyspnea, and pulmonary cysts. Annals of the American Thoracic Society, 12 1, 120-3Pasula, Rajamouli; Azad, Abul K; Gardner, Jason C; Schlesinger, Larry S; McCormack, Francis X 2015. Keratinocyte growth factor administration attenuates murine pulmonary mycobacterium tuberculosis infection through granulocyte-macrophage colony-stimulating factor (GM-CSF)-dependent macrophage activation and phagolysosome fusion. The Journal of biological chemistry, 290 11, 7151-9Quintanilla, Hector D; Liu, Yuying; Fatheree, Nicole Y; Atkins, Constance L; Hashmi, Syed S; Floros, Joanna; McCormack, Francis X; Rhoads, Jon Marc; Alcorn, Joseph L 2015. Oral administration of surfactant protein-a reduces pathology in an experimental model of necrotizing enterocolitis. Journal of pediatric gastroenterology and nutrition, 60 5, 613-20Saito, Atsushi; Nikolaidis, Nikolaos M; Amlal, Hassane; Uehara, Yasuaki; Gardner, Jason C; LaSance, Kathleen; Pitstick, Lori B; Bridges, James P; Wikenheiser-Brokamp, Kathryn A; McGraw, Dennis W; Woods, Jason C; Sabbagh, Yves; Schiavi, Susan C; Altini?ik, Göksel; Jakopovi?, Marko; Inoue, Yoshikazu; McCormack, Francis X 2015. Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment. Science translational medicine, 7 313, 313ra181Yang, Linlin; Carrillo, Marykate; Wu, Yuchieh M; DiAngelo, Susan L; Silveyra, Patricia; Umstead, Todd M; Halstead, E Scott; Davies, Michael L; Hu, Sanmei; Floros, Joanna; McCormack, Francis X; Christensen, Neil D; Chroneos, Zissis C 2015. SP-R210 (Myo18A) Isoforms as Intrinsic Modulators of Macrophage Priming and Activation. PloS one, 10 5, e0126576Cundiff, William B; McCormack, Francis X; Wikenheiser-Brokamp, Kathryn; Starnes, Sandra; Kotloff, Robert; Benzaquen, Sadia 2014. Successful management of a chronic, refractory bronchopleural fistula with endobronchial valves followed by talc pleurodesis. American journal of respiratory and critical care medicine, 189 4, 490-1Gardner, Jason C; Noel, John G; Nikolaidis, Nikolaos M; Karns, Rebekah; Aronow, Bruce J; Ogle, Cora K; McCormack, Francis X 2014. G-CSF drives a posttraumatic immune program that protects the host from infection. Journal of immunology (Baltimore, Md. : 1950), 192 5, 2405-17Gupta, R; Kitaichi, M; Inoue, Y; Kotloff, R; McCormack, F X 2014. Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology, 47 3, 106-17Gupta, R; Kitaichi, M; Inoue, Y; Kotloff, R; McCormack, F X 2014. Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology, 47 3, 106-17McCormack, Francis X 2014. Chronic sirolimus therapy for lymphangioleiomyomatosis. American journal of respiratory and critical care medicine, 190 12, 1332-3Nikolaidis, Nikolaos M; White, Mitchell R; Allen, Kimberly; Tripathi, Shweta; Qi, Li; McDonald, Barbara; Taubenberger, Jeffery; Seaton, Barbara A; McCormack, Francis X; Crouch, Erika C; Hartshorn, Kevan L 2014. Mutations flanking the carbohydrate binding site of surfactant protein D confer antiviral activity for pandemic influenza A viruses. American journal of physiology. Lung cellular and molecular physiology, 306 11, L1036-44Safi, Fadi; Gupta, Anita; Adams, Denise; Anandan, Vasuki; McCormack, Francis X; Assaly, Ragheb 2014. Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. Annals of the American Thoracic Society, 11 1, 92-5Cudzilo, Corey J; Szczesniak, Rhonda D; Brody, Alan S; Rattan, Mantosh S; Krueger, Darcy A; Bissler, John J; Franz, David N; McCormack, Francis X; Young, Lisa R 2013. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest, 144 2, 578-85Czyzyk-Krzeska, Maria F; McCormack, Francis X 2013. Birt-Hogg-Dubé syndrome. Familial cancer, 12 3, 355-6Gupta, Nishant; McCormack, Francis X 2013. Pulmonary alveolar microlithiasis. American journal of respiratory and critical care medicine, 188 7, e11-2Gupta, Nishant; Seyama, Kuniaki; McCormack, Francis X 2013. Pulmonary manifestations of Birt-Hogg-Dubé syndrome. Familial cancer, 12 3, 387-96Krueger, Darcy A; Northrup, Hope 2013. Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatric neurology, 49 4, 255-65McCormack, Francis X; Travis, William D; Colby, Thomas V; Henske, Elizabeth P; Moss, Joel 2013. Reply: Characterization of lymphangioleiomyomatosis as a neoplastic disease. American journal of respiratory and critical care medicine, 188 3, 398-9McCormack, Francis X; Wilmott, Clay; Young, Lisa R; Wikenheiser-Brokamp, Kathryn 2013. An atoll variant that mocks. American journal of respiratory and critical care medicine, 188 1, 110Northrup, Hope; Krueger, Darcy A 2013. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 Iinternational Tuberous Sclerosis Complex Consensus Conference. Pediatric neurology, 49 4, 243-54Oprescu, N; McCormack, F X; Byrnes, S; Kinder, B W 2013. Clinical predictors of mortality and cause of death in lymphangioleiomyomatosis: a population-based registry. Lung, 191 1, 35-42Safi, Fadi; Gupta, Anita; Adams, Denise; Anandan, Vasuki; McCormack, Francis X; Assaly, Ragheb 2013. Kaposiform Lymphangiomatosis, a Newly Characterized Vascular Anomaly Presenting with Hemoptysis in an Adult Woman. Annals of the American Thoracic Society, , Swigris, Jeffrey J; Lee, Hye-Seung; Cohen, Marsha; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Young, Lisa R; McCormack, Francis X 2013. St. George's Respiratory Questionnaire has longitudinal construct validity in lymphangioleiomyomatosis. Chest, 143 6, 1671-8Young, Lisa R; Lee, Hye-Seung; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Strange, Charlie; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Downey, Gregory P; Swigris, Jeffrey J; Taveira-Dasilva, Angelo M; Krischer, Jeffrey P; Trapnell, Bruce C; McCormack, Francis X 2013. Serum VEGF-D concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. The lancet. Respiratory medicine, 1 6, 445-452Zafar, Muhammad A; McCormack, Francis X; Rahman, Shahid; Tencza, Christian; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; Shizukuda, Yukitaka; Elwing, Jean M 2013. Pulmonary vascular shunts in exercise-intolerant patients with lymphangioleiomyomatosis. American journal of respiratory and critical care medicine, 188 9, 1167-70Henske, Elizabeth P; McCormack, Francis X 2012. Lymphangioleiomyomatosis - a wolf in sheep's clothing. The Journal of clinical investigation, 122 11, 3807-16McCormack, Francis X; Travis, William D; Colby, Thomas V; Henske, Elizabeth P; Moss, Joel 2012. Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. American journal of respiratory and critical care medicine, 186 12, 1210-2Meraj, Riffat; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; Byrnes, Sue; McCormack, Francis X 2012. Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis. Frontiers of medicine, 6 4, 395-405Meraj, Riffat; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; McCormack, Francis X 2012. Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Seminars in respiratory and critical care medicine, 33 5, 486-97Young, Lisa R; Gulleman, Peter M; Bridges, James P; Weaver, Timothy E; Deutsch, Gail H; Blackwell, Timothy S; McCormack, Francis X 2012. The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome. American journal of respiratory and critical care medicine, 186 10, 1014-24Crouch, Erika; Nikolaidis, Nikolaos; McCormack, Francis X; McDonald, Barbara; Allen, Kimberly; Rynkiewicz, Michael J; Cafarella, Tanya M; White, Mitchell; Lewnard, Kara; Leymarie, Nancy; Zaia, Joseph; Seaton, Barbara A; Hartshorn, Kevan L 2011. Mutagenesis of surfactant protein D informed by evolution and x-ray crystallography enhances defenses against influenza A virus in vivo. The Journal of biological chemistry, 286 47, 40681-92Hagaman, Jared T; Panos, Ralph J; McCormack, Francis X; Thakar, Charuhas V; Wikenheiser-Brokamp, Kathryn A; Shipley, Ralph T; Kinder, Brent W 2011. Vitamin D deficiency and reduced lung function in connective tissue-associated interstitial lung diseases. Chest, 139 2, 353-60McCormack, Francis X; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Strange, Charlie; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Young, Lisa R; Kinder, Brent W; Downey, Gregory P; Sullivan, Eugene J; Colby, Thomas V; McKay, Roy T; Cohen, Marsha M; Korbee, Leslie; Taveira-DaSilva, Angelo M; Lee, Hye-Seung; Krischer, Jeffrey P; Trapnell, Bruce C 2011. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. The New England journal of medicine, 364 17, 1595-606Seaman, Danielle M; Meyer, Cristopher A; Gilman, Matthew D; McCormack, Francis X 2011. Diffuse Cystic Lung Disease at High-Resolution CT. AJR. American journal of roentgenology, 196 6, 1305-11Shang, Feifei; Rynkiewicz, Michael J; McCormack, Francis X; Wu, Huixing; Cafarella, Tanya M; Head, James F; Seaton, Barbara A 2011. Crystallographic complexes of surfactant protein A and carbohydrates reveal ligand-induced conformational change. The Journal of biological chemistry, 286 1, 757-65Wu, Huixing; Suzuki, Takuji; Carey, Brenna; Trapnell, Bruce C; McCormack, Francis X 2011. Keratinocyte growth factor augments pulmonary innate immunity through epithelium-driven, GM-CSF-dependent paracrine activation of alveolar macrophages. The Journal of biological chemistry, 286 17, 14932-40Franz, D N; Bissler, J J; McCormack, F X 2010. Tuberous sclerosis complex: neurological, renal and pulmonary manifestations. Neuropediatrics, 41 5, 199-208Hagaman, Jared T; Ferguson, Bryce; Meyer, Cris; Wang, Jiang; McCormack, Francis X; Nath, Amaresh 2010. Endobronchial Carcinoid Tumor in an HIV-infected Patient. Journal of bronchology & interventional pulmonology, 17 2, 174-6Hagaman, Jared T; Schauer, Daniel P; McCormack, Francis X; Kinder, Brent W 2010. Screening for lymphangioleiomyomatosis by high-resolution computed tomography in young, nonsmoking women presenting with spontaneous pneumothorax is cost-effective. American journal of respiratory and critical care medicine, 181 12, 1376-82Johnson, S R; Cordier, J F; Lazor, R; Cottin, V; Costabel, U; Harari, S; Reynaud-Gaubert, M; Boehler, A; Brauner, M; Popper, H; Bonetti, F; Kingswood, C 2010. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. The European respiratory journal, 35 1, 14-26Kinder, Brent W; Sherman, A C; Young, L R; Hagaman, J T; Oprescu, N; Byrnes, S; McCormack, Francis X 2010. Predictors for clinical trial participation in the rare lung disease lymphangioleiomyomatosis. Respiratory medicine, 104 4, 578-83Kinder, Brent; McCormack, Francis X 2010. Clinical trials for rare lung diseases: lessons from lymphangioleiomyomatosis. Lymphatic research and biology, 8 1, 71-9Seaton, Barbara A; Crouch, Erika C; McCormack, Francis X; Head, James F; Hartshorn, Kevan L; Mendelsohn, Richard 2010. Review: Structural determinants of pattern recognition by lung collectins. Innate immunity, 16 3, 143-50Young, Lisa R; Vandyke, Rhonda; Gulleman, Peter M; Inoue, Yoshikazu; Brown, Kevin K; Schmidt, Laura S; Linehan, W Marston; Hajjar, Fuad; Kinder, Brent W; Trapnell, Bruce C; Bissler, John J; Franz, David N; McCormack, Francis X 2010. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest, 138 3, 674-81Gil, Malgorzata; McCormack, Francis X; Levine, Ann Marie 2009. Surfactant protein A modulates cell surface expression of CR3 on alveolar macrophages and enhances CR3-mediated phagocytosis. The Journal of biological chemistry, 284 12, 7495-504Kinder, Brent W; Brown, Kevin K; McCormack, Francis X; Ix, Joachim H; Kervitsky, Alma; Schwarz, Marvin I; King, Talmadge E 2009. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest, 135 6, 1557-63Schmithorst, Vincent J; Altes, Talissa A; Young, Lisa R; Franz, David N; Bissler, John J; McCormack, Francis X; Dardzinski, Bernard J; Brody, Alan S 2009. Automated algorithm for quantifying the extent of cystic change on volumetric chest CT: initial results in Lymphangioleiomyomatosis. AJR. American journal of roentgenology, 192 4, 1037-44Young, Lisa R; Franz, David N; Nagarkatte, Preeti; Fletcher, Christopher D M; Wikenheiser-Brokamp, Kathryn A; Galsky, Matthew D; Corbridge, Thomas C; Lam, Anna P; Gelfand, Michael J; McCormack, Francis X 2009. Utility of [18F]2-fluoro-2-deoxyglucose-PET in sporadic and tuberous sclerosis-associated lymphangioleiomyomatosis. Chest, 136 3, 926-33Ali, Naeem A; O'Brien, James M; Hoffmann, Stephen P; Phillips, Gary; Garland, Allan; Finley, James C W; Almoosa, Khalid; Hejal, Rana; Wolf, Karen M; Lemeshow, Stanley; Connors, Alfred F; Marsh, Clay B 2008. Acquired weakness, handgrip strength, and mortality in critically ill patients. American journal of respiratory and critical care medicine, 178 3, 261-8Bissler, John J; McCormack, Francis X; Young, Lisa R; Elwing, Jean M; Chuck, Gail; Leonard, Jennifer M; Schmithorst, Vincent J; Laor, Tal; Brody, Alan S; Bean, Judy; Salisbury, Shelia; Franz, David N 2008. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. The New England journal of medicine, 358 2, 140-51McCormack, Francis X 2008. Lymphangioleiomyomatosis: a clinical update. Chest, 133 2, 507-16Young, Lisa R; Inoue, Yoshikazu; McCormack, Francis X 2008. Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis. The New England journal of medicine, 358 2, 199-200Byrnes, Abigail B; McCormack, Francis X; Diers, Tiffiny; Jazieh, Abdul-Rahman 2007. The resident scholar program: a research training opportunity for internal medicine house staff. Journal of cancer education : the official journal of the American Association for Cancer Education, 22 1, 47-9Juvet, Stephen C; McCormack, Francis X; Kwiatkowski, David J; Downey, Gregory P 2007. Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphans. American journal of respiratory cell and molecular biology, 36 4, 398-408McCormack, Francis X; Moss, Joel 2007. S-LAM in a man? American journal of respiratory and critical care medicine, 176 1, 3-5Pollock-BarZiv, Stacey; Cohen, Marsha M; Downey, Gregory P; Johnson, Simon R; Sullivan, Eugene; McCormack, Francis X 2007. Air travel in women with lymphangioleiomyomatosis. Thorax, 62 2, 176-80Young, Lisa R; Pasula, Rajamouli; Gulleman, Peter M; Deutsch, Gail H; McCormack, Francis X 2007. Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosis. American journal of respiratory cell and molecular biology, 37 1, 67-74Zhang, Shiping; McCormack, Francis X; Levesque, Roger C; O'Toole, George A; Lau, Gee W 2007. The flagellum of Pseudomonas aeruginosa is required for resistance to clearance by surfactant protein A. PloS one, 2 6, e564Almoosa, Khalid F; McCormack, Francis X; Sahn, Steven A 2006. Pleural disease in lymphangioleiomyomatosis. Clinics in chest medicine, 27 2, 355-68Almoosa, Khalid F; Ryu, Jay H; Mendez, Jose; Huggins, J Terrill; Young, Lisa R; Sullivan, Eugene J; Maurer, Janet; McCormack, Francis X; Sahn, Steven A 2006. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest, 129 5, 1274-81Kingma, Paul S; Zhang, Liqian; Ikegami, Machiko; Hartshorn, Kevan; McCormack, Francis X; Whitsett, Jeffrey A 2006. Correction of pulmonary abnormalities in Sftpd-/- mice requires the collagenous domain of surfactant protein D. The Journal of biological chemistry, 281 34, 24496-505Kuzmenko, Alexander I; Wu, Huixing; McCormack, Francis X 2006. Pulmonary collectins selectively permeabilize model bacterial membranes containing rough lipopolysaccharide. Biochemistry, 45 8, 2679-85McCormack, Francis X 2006. Lymphangioleiomyomatosis. MedGenMed : Medscape general medicine, 8 1, 15McCormack, Francis X 2006. New concepts in collectin-mediated host defense at the air-liquid interface of the lung. Respirology (Carlton, Vic.), 11 Suppl , S7-10Ryan, Marnie A; Akinbi, Henry T; Serrano, Alicia G; Perez-Gil, Jesus; Wu, Huixing; McCormack, Francis X; Weaver, Timothy E 2006. Antimicrobial activity of native and synthetic surfactant protein B peptides. Journal of immunology (Baltimore, Md. : 1950), 176 1, 416-25Ryu, Jay H; Moss, Joel; Beck, Gerald J; Lee, Jar-Chi; Brown, Kevin K; Chapman, Jeffrey T; Finlay, Geraldine A; Olson, Eric J; Ruoss, Stephen J; Maurer, Janet R; Raffin, Thomas A; Peavy, Hannah H; McCarthy, Kevin; Taveira-Dasilva, Angelo; McCormack, Francis X; Avila, Nilo A; Decastro, Rosamma M; Jacobs, Susan S; Stylianou, Mario; Fanburg, Barry L 2006. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. American journal of respiratory and critical care medicine, 173 1, 105-11Schlosser, A; Thomsen, T; Shipley, J M; Hein, P W; Brasch, F; Tornøe, I; Nielsen, O; Skjødt, K; Palaniyar, N; Steinhilber, W; McCormack, F X; Holmskov, U 2006. Microfibril-associated protein 4 binds to surfactant protein A (SP-A) and colocalizes with SP-A in the extracellular matrix of the lung. Scandinavian journal of immunology, 64 2, 104-16Young, Lisa R; Almoosa, Khalid F; Pollock-Barziv, Stacey; Coutinho, Meg; McCormack, Francis X; Sahn, Steven A 2006. Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis. Chest, 129 5, 1267-73Young, Lisa R; Borchers, Michael T; Allen, Holly L; Gibbons, Reta S; McCormack, Francis X 2006. Lung-restricted macrophage activation in the pearl mouse model of Hermansky-Pudlak syndrome. Journal of immunology (Baltimore, Md. : 1950), 176 7, 4361-8Zhang, Liqian; Ikegami, Machiko; Korfhagen, Thomas R; McCormack, Francis X; Yoshida, Mitsuhiro; Senior, Robert M; Shipley, J Michael; Shapiro, Steven D; Whitsett, Jeffrey A 2006. Neither SP-A nor NH2-terminal domains of SP-A can substitute for SP-D in regulation of alveolar homeostasis. American journal of physiology. Lung cellular and molecular physiology, 291 2, L181-90Beharka, Alison A; Crowther, Joy E; McCormack, Francis X; Denning, Gerene M; Lees, Jason; Tibesar, Eric; Schlesinger, Larry S 2005. Pulmonary surfactant protein A activates a phosphatidylinositol 3-kinase/calcium signal transduction pathway in human macrophages: participation in the up-regulation of mannose receptor activity. Journal of immunology (Baltimore, Md. : 1950), 175 4, 2227-36Kuzmenko, Alexander I; Wu, Huixing; Wan, Sijue; McCormack, Francis X 2005. Surfactant protein A is a principal and oxidation-sensitive microbial permeabilizing factor in the alveolar lining fluid. The Journal of biological chemistry, 280 27, 25913-9Zhang, Shiping; Chen, Yi; Potvin, Eric; Sanschagrin, Francois; Levesque, Roger C; McCormack, Francis X; Lau, Gee W 2005. Comparative signature-tagged mutagenesis identifies Pseudomonas factors conferring resistance to the pulmonary collectin SP-A. PLoS pathogens, 1 3, 259-68Crowther, Joy E; Kutala, Vijay Kumar; Kuppusamy, Periannan; Ferguson, J Scott; Beharka, Alison A; Zweier, Jay L; McCormack, Francis X; Schlesinger, Larry S 2004. Pulmonary surfactant protein a inhibits macrophage reactive oxygen intermediate production in response to stimuli by reducing NADPH oxidase activity. Journal of immunology (Baltimore, Md. : 1950), 172 11, 6866-74Kuzmenko, A I; Wu, H; Bridges, J P; McCormack, F X 2004. Surfactant lipid peroxidation damages surfactant protein A and inhibits interactions with phospholipid vesicles. Journal of lipid research, 45 6, 1061-8Schaeffer, Lyndsay M; McCormack, Francis X; Wu, Huixing; Weiss, Alison A 2004. Interactions of pulmonary collectins with Bordetella bronchiseptica and Bordetella pertussis lipopolysaccharide elucidate the structural basis of their antimicrobial activities. Infection and immunity, 72 12, 7124-30Schaeffer, Lyndsay M; McCormack, Francis X; Wu, Huixing; Weiss, Alison A 2004. Bordetella pertussis lipopolysaccharide resists the bactericidal effects of pulmonary surfactant protein A. Journal of immunology (Baltimore, Md. : 1950), 173 3, 1959-65Chabot, Sophie; Salez, Laurent; McCormack, Francis X; Touqui, Lhousseine; Chignard, Michel 2003. Surfactant protein A inhibits lipopolysaccharide-induced in vivo production of interleukin-10 by mononuclear phagocytes during lung inflammation. American journal of respiratory cell and molecular biology, 28 3, 347-53Head, James F; Mealy, Tanya R; McCormack, Francis X; Seaton, Barbara A 2003. Crystal structure of trimeric carbohydrate recognition and neck domains of surfactant protein A. The Journal of biological chemistry, 278 44, 43254-60Karbowniczek, Magdalena; Astrinidis, Aristotelis; Balsara, Binaifer R; Testa, Joseph R; Lium, James H; Colby, Thomas V; McCormack, Francis X; Henske, Elizabeth Petri 2003. Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism. American journal of respiratory and critical care medicine, 167 7, 976-82McCormack, Francis X; Gibbons, Reta; Ward, Susan R; Kuzmenko, Alexander; Wu, Huixing; Deepe, George S 2003. Macrophage-independent fungicidal action of the pulmonary collectins. The Journal of biological chemistry, 278 38, 36250-6Rodriguez Capote, Karina; McCormack, Francis X; Possmayer, Fred 2003. Pulmonary surfactant protein-A (SP-A) restores the surface properties of surfactant after oxidation by a mechanism that requires the Cys6 interchain disulfide bond and the phospholipid binding domain. The Journal of biological chemistry, 278 23, 20461-74Sietsema, Kathy E; McCormack, Francis X 2003. Lymphangioleiomyomatosis: insights about, and from, a rare disease. American journal of respiratory and critical care medicine, 168 12, 1405-6Wu, Huixing; Kuzmenko, Alexander; Wan, Sijue; Schaffer, Lyndsay; Weiss, Alison; Fisher, James H; Kim, Kwang Sik; McCormack, Francis X 2003. Surfactant proteins A and D inhibit the growth of Gram-negative bacteria by increasing membrane permeability. The Journal of clinical investigation, 111 10, 1589-602Beharka, Alison A; Gaynor, Cecilia D; Kang, Byoung K; Voelker, Dennis R; McCormack, Francis X; Schlesinger, Larry S 2002. Pulmonary surfactant protein A up-regulates activity of the mannose receptor, a pattern recognition receptor expressed on human macrophages. Journal of immunology (Baltimore, Md. : 1950), 169 7, 3565-73Guillot, Loïc; Balloy, Viviane; McCormack, Francis X; Golenbock, Douglas T; Chignard, Michel; Si-Tahar, Mustapha 2002. Cutting edge: the immunostimulatory activity of the lung surfactant protein-A involves Toll-like receptor 4. Journal of immunology (Baltimore, Md. : 1950), 168 12, 5989-92Malloy, Jaret L; Veldhuizen, Ruud A W; McCormack, Francis X; Korfhagen, Thomas R; Whitsett, Jeffery A; Lewis, James F 2002. Pulmonary surfactant and inflammation in septic adult mice: role of surfactant protein A. Journal of applied physiology (Bethesda, Md. : 1985), 92 2, 809-16McCormack, Francis X; Whitsett, Jeffrey A 2002. The pulmonary collectins, SP-A and SP-D, orchestrate innate immunity in the lung. The Journal of clinical investigation, 109 6, 707-12McCormack, Francis; Brody, Alan; Meyer, Christopher; Leonard, Jennifer; Chuck, Gail; Dabora, Sandra; Sethuraman, Gopalan; Colby, Thomas V; Kwiatkowski, David J; Franz, David N 2002. Pulmonary cysts consistent with lymphangioleiomyomatosis are common in women with tuberous sclerosis: genetic and radiographic analysis. Chest, 121 3 Suppl, 61SPalaniyar, Nades; Zhang, Liquian; Kuzmenko, Alexander; Ikegami, Machiko; Wan, Sijue; Wu, Huixing; Korfhagen, Thomas R; Whitsett, Jeffrey A; McCormack, Francis X 2002. The role of pulmonary collectin N-terminal domains in surfactant structure, function, and homeostasis in vivo. The Journal of biological chemistry, 277 30, 26971-9Franz, D N; Brody, A; Meyer, C; Leonard, J; Chuck, G; Dabora, S; Sethuraman, G; Colby, T V; Kwiatkowski, D J; McCormack, F X 2001. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. American journal of respiratory and critical care medicine, 164 4, 661-8Ikegami, M; Elhalwagi, B M; Palaniyar, N; Dienger, K; Korfhagen, T; Whitsett, J A; McCormack, F X 2001. The collagen-like region of surfactant protein A (SP-A) is required for correction of surfactant structural and functional defects in the SP-A null mouse. The Journal of biological chemistry, 276 42, 38542-8Linke, M J; Harris, C E; Korfhagen, T R; McCormack, F X; Ashbaugh, A D; Steele, P; Whitsett, J A; Walzer, P D 2001. Immunosuppressed surfactant protein A-deficient mice have increased susceptibility to Pneumocystis carinii infection. The Journal of infectious diseases, 183 6, 943-52Palaniyar, N; Ikegami, M; Korfhagen, T; Whitsett, J; McCormack, F X 2001. Domains of surfactant protein A that affect protein oligomerization, lipid structure and surface tension. Comparative biochemistry and physiology. Part A, Molecular & integrative physiology, 129 1, 109-27Salez, L; Balloy, V; van Rooijen, N; Lebastard, M; Touqui, L; McCormack, F X; Chignard, M 2001. Surfactant protein A suppresses lipopolysaccharide-induced IL-10 production by murine macrophages. Journal of immunology (Baltimore, Md. : 1950), 166 10, 6376-82Bridges, J P; Davis, H W; Damodarasamy, M; Kuroki, Y; Howles, G; Hui, D Y; McCormack, F X 2000. Pulmonary surfactant proteins A and D are potent endogenous inhibitors of lipid peroxidation and oxidative cellular injury. The Journal of biological chemistry, 275 49, 38848-55Damodarasamy, M; Zhang, M; Dienger, K; McCormack, F X 2000. Two rat surfactant protein A isoforms arise by a novel mechanism that includes alternative translation initiation. Biochemistry, 39 33, 10189-95Fisher, J H; Sheftelyevich, V; Ho, Y S; Fligiel, S; McCormack, F X; Korfhagen, T R; Whitsett, J A; Ikegami, M 2000. Pulmonary-specific expression of SP-D corrects pulmonary lipid accumulation in SP-D gene-targeted mice. American journal of physiology. Lung cellular and molecular physiology, 278 2, L365-73Palaniyar, N; McCormack, F X; Possmayer, F; Harauz, G 2000. Three-dimensional structure of rat surfactant protein A trimers in association with phospholipid monolayers. Biochemistry, 39 21, 6310-6Elhalwagi, B M; Zhang, M; Ikegami, M; Iwamoto, H S; Morris, R E; Miller, M L; Dienger, K; McCormack, F X 1999. Normal surfactant pool sizes and inhibition-resistant surfactant from mice that overexpress surfactant protein A. American journal of respiratory cell and molecular biology, 21 3, 380-7Ferguson, J S; Voelker, D R; McCormack, F X; Schlesinger, L S 1999. Surfactant protein D binds to Mycobacterium tuberculosis bacilli and lipoarabinomannan via carbohydrate-lectin interactions resulting in reduced phagocytosis of the bacteria by macrophages. Journal of immunology (Baltimore, Md. : 1950), 163 1, 312-21McCormack, F X; Damodarasamy, M; Elhalwagi, B M 1999. Deletion mapping of N-terminal domains of surfactant protein A. The N-terminal segment is required for phospholipid aggregation and specific inhibition of surfactant secretion. The Journal of biological chemistry, 274 5, 3173-81Yu, S H; McCormack, F X; Voelker, D R; Possmayer, F 1999. Interactions of pulmonary surfactant protein SP-A with monolayers of dipalmitoylphosphatidylcholine and cholesterol: roles of SP-A domains. 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The European respiratory journal, 57 6, Foley, M; Duffy, F; Skally, M; McCormack, F; Finn, C; O'Connor, M; Cafferkey, J; Thomas, T; Burns, K; Fitzpatrick, F; O'Connell, K; Smyth, E G; Humphreys, H 2021. Evolving epidemiology of carbapenemase-producing Enterobacterales: one hospital's infection prevention and control response over nine years. The Journal of hospital infection, 112 , 61-68McCarthy, Cormac; Gupta, Nishant; Johnson, Simon R; Yu, Jane J; McCormack, Francis X 2021. Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management. The Lancet. Respiratory medicine, , Northrup, Hope; Aronow, Mary E; Bebin, E Martina; Bissler, John; Darling, Thomas N; de Vries, Petrus J; Frost, Michael D; Fuchs, Zoë; Gosnell, Elizabeth S; Gupta, Nishant; Jansen, Anna C; Jó?wiak, Sergiusz; Kingswood, J Chris; Knilans, Timothy K; McCormack, Francis X; Pounders, Ashley; Roberds, Steven L; Rodriguez-Buritica, David F; Roth, Jonathan; Sampson, Julian R; Sparagana, Steven; Thiele, Elizabeth Anne; Weiner, Howard L; Wheless, James W; Towbin, Alexander J; Krueger, Darcy A 2021. Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations. Pediatric neurology, 123 , 50-66Tian, Xinlun; Glass, Jennifer E; Kwiatkowski, David J; Towbin, Alexander J; Li, Yinan; Sund, Kristen L; Krueger, Darcy A; Franz, David N; McCormack, Francis X; Gupta, Nishant 2021. Lymphangioleiomyomatosis Association with Underlying Genotype in Patients with Tuberous Sclerosis Complex. Annals of the American Thoracic Society, 18 5, 815-819White, Mitchell R; Nikolaidis, Nikolaos M; McCormack, Francis; Crouch, Erika C; Hartshorn, Kevan L 2021. Viral Evasion of Innate Immune Defense: The Case of Resistance of Pandemic H1N1 Influenza A Virus to Human Mannose-Binding Proteins. Frontiers in microbiology, 12 , 774711Dellinger, Michael T; McCormack, Francis X 2020. The emergence of targetable MEKanisms in sporadic lymphatic disorders. EMBO molecular medicine, , e12822Guo, Minzhe; Yu, Jane J; Perl, Anne Karina; Wikenheiser-Brokamp, Kathryn A; Riccetti, Matt; Zhang, Erik Y; Sudha, Parvathi; Adam, Mike; Potter, Andrew; Kopras, Elizabeth J; Giannikou, Krinio; Potter, S Steven; Sherman, Sue; Hammes, Stephen R; Kwiatkowski, David J; Whitsett, Jeffrey A; McCormack, Francis X; Xu, Yan 2020. Single Cell Transcriptomic Analysis Identifies a Unique Pulmonary Lymphangioleiomyomatosis Cell. American journal of respiratory and critical care medicine, , Gupta, Nishant; Bleesing, Jack H; McCormack, Francis X 2020. Successful Response to Treatment with Sirolimus in Pulmonary Sarcoidosis. American journal of respiratory and critical care medicine, , Gupta, Nishant; Hagner, Miles; Wu, Huixing; Young, Lisa R; Palipana, Anushka; Szczesniak, Rhonda D; McCormack, Francis X 2020. Serum Vascular Endothelial Growth Factor-C as a Marker of Therapeutic Response to Sirolimus in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Jönsson, Åsa Lina M; Bendstrup, Elisabeth; Mogensen, Susie; Kopras, Elizabeth J; McCormack, Francis X; Campo, Ilaria; Mariani, Francesca; Escribano-Montaner, Amparo; Holm, Are M; Martinez-Colls, Maria Del Mar; Pintos-Morell, Guillem; Taillé, Camille; Crestani, Bruno; Hilberg, Ole; Hvarregaard Christensen, Jane; Simonsen, Ulf 2020. Eight novel variants in the SLC34A2 gene in pulmonary alveolar microlithiasis. The European respiratory journal, 55 2, Kosciuk, Patrick; Meyer, Cristopher; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2020. Pulmonary alveolar microlithiasis. European respiratory review : an official journal of the European Respiratory Society, 29 158, Krymskaya, Vera P; Courtwright, Andrew M; Fleck, Victoria; Dorgan, Daniel; Kotloff, Robert; McCormack, Francis X; Kreider, Maryl 2020. A phase II clinical trial of the Safety Of Simvastatin (SOS) in patients with pulmonary lymphangioleiomyomatosis and with tuberous sclerosis complex. Respiratory medicine, 163 , 105898Liu, Huan; Osterburg, Andrew R; Flury, Jennifer; Swank, Zulma; McGraw, Dennis W; Gupta, Nishant; Wikenheiser-Brokamp, Kathryn A; Kumar, Ashish; Tazi, Abdellatif; Inoue, Yoshikazu; Hirose, Masaki; McCormack, Francis X; Borchers, Michael T 2020. MAPK mutations and cigarette smoke promote the pathogenesis of pulmonary Langerhans cell histiocytosis. JCI insight, 5 4, Poovelikunnel, Toney T; Duffy, Fionnuala; Puthussery, Thressia; Gangadharan, Shaini; McCormack, Fiona; Carpenter, Helen; Kizhakedath, Mary; Hawkshaw, Susan 2020. Clinically indicated replacement of peripheral vascular catheters: is it safe for patients? British journal of nursing (Mark Allen Publishing), 29 8, S4-S10Shaw, Brian M; Shaw, Steven D; McCormack, Francis X 2020. Pulmonary Alveolar Microlithiasis. Seminars in respiratory and critical care medicine, 41 2, 280-287Gupta, Nishant; Lee, Hye-Seung; Ryu, Jay H; Taveira-DaSilva, Angelo M; Beck, Gerald J; Lee, Jar-Chi; McCarthy, Kevin; Finlay, Geraldine A; Brown, Kevin K; Ruoss, Stephen J; Avila, Nilo A; Moss, Joel; McCormack, Francis X 2019. The NHLBI LAM Registry: Prognostic Physiologic and Radiologic Biomarkers Emerge From a 15-Year Prospective Longitudinal Analysis. Chest, 155 2, 288-296Gupta, Nishant; Lee, Hye-Seung; Young, Lisa R; Strange, Charlie; Moss, Joel; Singer, Lianne G; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Downey, Gregory P; Taveira-DaSilva, Angelo M; Krischer, Jeffrey P; Setchell, Kenneth; Trapnell, Bruce C; Inoue, Yoshikazu; McCormack, Francis X 2019. Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis. The European respiratory journal, 53 4, Hirose, Masaki; Matsumuro, Akiko; Arai, Toru; Sugimoto, Chikatoshi; Akira, Masanori; Kitaichi, Masanori; Young, Lisa R; McCormack, Francis X; Inoue, Yoshikazu 2019. Serum vascular endothelial growth factor-D as a diagnostic and therapeutic biomarker for lymphangioleiomyomatosis. PloS one, 14 2, e0212776McCarthy, Cormac; Bartholmai, Brian J; Woods, Jason C; McCormack, Francis X; Trapnell, Bruce C 2019. Automated Parenchymal Pattern Analysis of Treatment Responses in Pulmonary Alveolar Proteinosis. American journal of respiratory and critical care medicine, , McCarthy, Cormac; McCormack, Francis X 2019. Recurrent Sinusitis, Lower Limb Edema, and Nail Changes. Annals of the American Thoracic Society, 16 6, 752-755Nalian, Armen; Umstead, Todd M; Yang, Ching-Hui; Silveyra, Patricia; Thomas, Neal J; Floros, Joanna; McCormack, Francis X; Chroneos, Zissis C 2019. Structural and Functional Determinants of Rodent and Human Surfactant Protein A: A Synthesis of Binding and Computational Data. Frontiers in immunology, 10 , 2613Walkup, Laura L; Roach, David J; Hall, Chase S; Gupta, Nishant; Thomen, Robert P; Cleveland, Zackary I; McCormack, Francis X; Woods, Jason C 2019. Cyst Ventilation Heterogeneity and Alveolar Airspace Dilation as Early Disease Markers in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 16 8, 1008-1016Zak, Sara; Mokhallati, Nadine; Su, Weiji; McCormack, Francis X; Franz, David N; Mays, Maxwell; Krueger, Darcy A; Szczesniak, Rhonda D; Gupta, Nishant 2019. Lymphangioleiomyomatosis Mortality in Patients with Tuberous Sclerosis Complex. Annals of the American Thoracic Society, 16 4, 509-512Gupta, Nishant; Colby, Thomas V; Meyer, Cristopher A; McCormack, Francis X; Wikenheiser-Brokamp, Kathryn A 2018. Smoking-Related Diffuse Cystic Lung Disease. Chest, 154 2, e31-e35Gupta, Nishant; Johnson, Simon R; Moss, Joel; McCormack, Francis X 2018. Reply to Yanagisawa: Treatment of Pulmonary Lymphangioleiomyomatosis during Pregnancy. American journal of respiratory and critical care medicine, 197 11, 1507-1508Liu, Huan; Osterburg, Andrew R; Flury, Jennifer; Huang, Shuo; McCormack, Francis X; Cormier, Stephania A; Borchers, Michael T 2018. NKG2D Regulation of Lung Pathology and Dendritic Cell Function Following Respiratory Syncytial Virus Infection. The Journal of infectious diseases, 218 11, 1822-1832Miller, Suzanne; Coveney, Clare; Johnson, Janice; Farmaki, Aliki-Eleni; Gupta, Nishant; Tobin, Martin D; Wain, Louise V; McCormack, Francis X; Boocock, David J; Johnson, Simon R 2018. The vitamin D binding protein axis modifies disease severity in lymphangioleiomyomatosis. The European respiratory journal, 52 5, Feemster, Laura C; Lyons, Patrick G; Chatterjee, Rohini S; Kidambi, Pranav; McCormack, Francis X; Moss, Joel; Wilson, Kevin C; Thomson, Carey C 2017. Summary for Clinicians: Lymphangioleiomyomatosis Diagnosis and Management Clinical Practice Guideline. Annals of the American Thoracic Society, 14 7, 1073-1075Gupta, Nishant; Finlay, Geraldine A; Kotloff, Robert M; Strange, Charlie; Wilson, Kevin C; Young, Lisa R; Taveira-DaSilva, Angelo M; Johnson, Simon R; Cottin, Vincent; Sahn, Steven A; Ryu, Jay H; Seyama, Kuniaki; Inoue, Yoshikazu; Downey, Gregory P; Han, MeiLan K; Colby, Thomas V; Wikenheiser-Brokamp, Kathryn A; Meyer, Cristopher A; Smith, Karen; Moss, Joel; McCormack, Francis X 2017. Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. American journal of respiratory and critical care medicine, 196 10, 1337-1348Gupta, Nishant; Kopras, Elizabeth J; Henske, Elizabeth P; James, Laura E; El-Chemaly, Souheil; Veeraraghavan, Srihari; Drake, Matthew G; McCormack, Francis X 2017. Spontaneous Pneumothoraces in Patients with Birt-Hogg-Dubé Syndrome. Annals of the American Thoracic Society, 14 5, 706-713Gupta, Nishant; Langenderfer, Dale; McCormack, Francis X; Schauer, Daniel P; Eckman, Mark H 2017. Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective. Annals of the American Thoracic Society, 14 1, 17-25Han, MeiLan K; Tyburczy, Magdalena E; Darling, Thomas N; Kazerooni, Ella A; Myers, Jeffrey L; McCormack, Francis X; Moss, Joel; Kwiatkowski, David J 2017. Apparent Sporadic Lymphangioleiomyomatosis (LAM) in a Man Due to Extreme Mosaicism for a TSC2 Mutation. Annals of the American Thoracic Society, , Kristof, Arnold S; Petrof, Basil J; Hamid, Qutayba; Kolb, Martin; Landry, Jennifer S; MacKenzie, Alex; McCormack, Francis X; Murawski, Inga J; Moss, Joel; Rauch, Frank; Rosas, Ivan O; Shapiro, Adam J; Smith, Benjamin M; Thomas, David Y; Trapnell, Bruce C; Young, Lisa R; Zariwala, Maimoona A 2017. An Official American Thoracic Society Workshop Report: Translational Research in Rare Respiratory Diseases. Annals of the American Thoracic Society, 14 8, 1239-1247Krymskaya, Vera P; McCormack, Francis X 2017. Lymphangioleiomyomatosis: A Monogenic Model of Malignancy. Annual review of medicine, 68 , 69-83Lee, Lynn H; Gasilina, Anjelika; Roychoudhury, Jayeeta; Clark, Jason; McCormack, Francis X; Pressey, Joseph; Grimley, Michael S; Lorsbach, Robert; Ali, Siraj; Bailey, Mark; Stephens, Philip; Ross, Jeffrey S; Miller, Vincent A; Nassar, Nicolas N; Kumar, Ashish R 2017. Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while improving treatment outcomes. JCI insight, 2 3, e89473Liu, Huan; Jakubzick, Claudia; Osterburg, Andrew R; Nelson, Rebecca L; Gupta, Nishant; McCormack, Francis X; Borchers, Michael T 2017. Dendritic Cell Trafficking and Function in Rare Lung Diseases. American journal of respiratory cell and molecular biology, , Lu, Calvin; Lee, Hye-Seung; Pappas, George P; Dilling, Daniel F; Burger, Charles D; Shifren, Adrian; Veeraraghavan, Srihari; Chapman, Jeffrey T; Parambil, Joseph; Ruoss, Stephen J; Young, Lisa R; Hammes, Stephen R; Kopras, Elizabeth J; Roads, Tammy; Krischer, Jeffrey P; McCormack, Francis X 2017. A Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 14 6, 919-928McCarthy, Cormac; Lara Gallego, Beatriz; Trapnell, Bruce C; McCormack, Francis X 2017. Epidemiology of Rare Lung Diseases: The Challenges and Opportunities to Improve Research and Knowledge. Advances in experimental medicine and biology, 1031 , 419-442McCormack, Francis X; Gupta, Nishant; Finlay, Geraldine; Young, Lisa R; Johnson, Simon; Cottin, Vincent; Wilson, Kevin; Moss, Joel 2017. Reply to Official ATS/JRS Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. American journal of respiratory and critical care medicine, , Nikolaidis, Nikolaos M; Noel, John G; Pitstick, Lori B; Gardner, Jason C; Uehara, Yasuaki; Wu, Huixing; Saito, Atsushi; Lewnard, Kara E; Liu, Huan; White, Mitchell R; Hartshorn, Kevan L; McCormack, Francis X 2017. Mitogenic stimulation accelerates influenza-induced mortality by increasing susceptibility of alveolar type II cells to infection. Proceedings of the National Academy of Sciences of the United States of America, 114 32, E6613-E6622Noel, John G; Ramser, Benjamin J; Cancelas, Jose A; McCormack, Francis X; Gardner, Jason C 2017. Thermal injury of the skin induces G-CSF-dependent attenuation of EPO-mediated STAT signaling and erythroid differentiation arrest in mice. Experimental hematology, , Rynkiewicz, Michael J; Wu, Huixing; Cafarella, Tanya R; Nikolaidis, Nikolaos M; Head, James F; Seaton, Barbara A; McCormack, Francis X 2017. Differential Ligand Binding Specificities of the Pulmonary Collectins Are Determined by the Conformational Freedom of a Surface Loop. Biochemistry, 56 31, 4095-4105Sontake, Vishwaraj; Wang, Yunguan; Kasam, Rajesh K; Sinner, Debora; Reddy, Geereddy B; Naren, Anjaparavanda P; McCormack, Francis X; White, Eric S; Jegga, Anil G; Madala, Satish K 2017. Hsp90 regulation of fibroblast activation in pulmonary fibrosis. JCI insight, 2 4, e91454Wang, Yunguan; Yella, Jaswanth; Chen, Jing; McCormack, Francis X; Madala, Satish K; Jegga, Anil G 2017. Unsupervised gene expression analyses identify IPF-severity correlated signatures, associated genes and biomarkers. BMC pulmonary medicine, 17 1, 133Argula, Rahul G; Kokosi, Maria; Lo, Pechin; Kim, Hyun J; Ravenel, James G; Meyer, Cristopher; Goldin, Jonathan; Lee, Hye-Seung; Strange, Charlie; McCormack, Francis X 2016. A Novel Quantitative Computed Tomographic Analysis Suggests How Sirolimus Stabilizes Progressive Air Trapping in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 13 3, 342-9Gardner, Jason C; Wu, Huixing; Noel, John G; Ramser, Benjamin J; Pitstick, Lori; Saito, Atsushi; Nikolaidis, Nikolaos M; McCormack, Francis X 2016. Keratinocyte growth factor supports pulmonary innate immune defense through maintenance of alveolar antimicrobial protein levels and macrophage function. American journal of physiology. Lung cellular and molecular physiology, 310 9, L868-79Goh, Boon Chong; Wu, Huixing; Rynkiewicz, Michael J; Schulten, Klaus; Seaton, Barbara A; McCormack, Francis X 2016. Elucidation of Lipid Binding Sites on Lung Surfactant Protein A Using X-ray Crystallography, Mutagenesis, and Molecular Dynamics Simulations. Biochemistry, 55 26, 3692-701Gupta, Nishant; Wikenheiser-Brokamp, Kathryn A; Fischer, Aryeh; McCormack, Francis X 2016. Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren Syndrome. Annals of the American Thoracic Society, 13 3, 371-5Itkin, Maxim G; McCormack, Francis X; Dori, Yoav 2016. Diagnosis and Treatment of Lymphatic Plastic Bronchitis in Adults Using Advanced Lymphatic Imaging and Percutaneous Embolization. Annals of the American Thoracic Society, 13 10, 1689-1696Itkin, Maxim; McCormack, Francis X 2016. Nonmalignant Adult Thoracic Lymphatic Disorders. Clinics in chest medicine, 37 3, 409-20Kotloff, Robert M; McCormack, Francis X 2016. Rare Lung Diseases: Occasionally the "Horse" Has Stripes. Clinics in chest medicine, 37 3, xv-xviMcCormack, Francis X; Gupta, Nishant; Finlay, Geraldine R; Young, Lisa R; Taveira-DaSilva, Angelo M; Glasgow, Connie G; Steagall, Wendy K; Johnson, Simon R; Sahn, Steven A; Ryu, Jay H; Strange, Charlie; Seyama, Kuniaki; Sullivan, Eugene J; Kotloff, Robert M; Downey, Gregory P; Chapman, Jeffrey T; Han, MeiLan K; D'Armiento, Jeanine M; Inoue, Yoshikazu; Henske, Elizabeth P; Bissler, John J; Colby, Thomas V; Kinder, Brent W; Wikenheiser-Brokamp, Kathryn A; Brown, Kevin K; Cordier, Jean F; Meyer, Cristopher; Cottin, Vincent; Brozek, Jan L; Smith, Karen; Wilson, Kevin C; Moss, Joel 2016. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management. American journal of respiratory and critical care medicine, 194 6, 748-61Osterburg, Andrew R; Nelson, Rebecca L; Yaniv, Benyamin Z; Foot, Rachel; Donica, Walter R F; Nashu, Madison A; Liu, Huan; Wikenheiser-Brokamp, Kathryn A; Moss, Joel; Gupta, Nishant; McCormack, Francis X; Borchers, Michael T 2016. NK cell activating receptor ligand expression in lymphangioleiomyomatosis is associated with lung function decline. JCI insight, 1 16, e87270Saito, Atsushi; McCormack, Francis X 2016. Pulmonary Alveolar Microlithiasis. Clinics in chest medicine, 37 3, 441-8Soleimani, Manoocher; Barone, Sharon; Xu, Jie; Alshahrani, Saeed; Brooks, Marybeth; McCormack, Francis X; Smith, Roger D; Zahedi, Kamyar 2016. Prostaglandin-E2 Mediated Increase in Calcium and Phosphate Excretion in a Mouse Model of Distal Nephron Salt Wasting. PloS one, 11 7, e0159804Takada, Toshinori; Mikami, Ayako; Kitamura, Nobutaka; Seyama, Kuniaki; Inoue, Yoshikazu; Nagai, Katsura; Suzuki, Masaru; Moriyama, Hiroshi; Akasaka, Keiichi; Tazawa, Ryushi; Hirai, Toyohiro; Mishima, Michiaki; Hayashida, Mie; Hirose, Masaki; Sugimoto, Chikatoshi; Arai, Toru; Hattori, Noboru; Watanabe, Kentaro; Tamada, Tsutomu; Yoshizawa, Hirohisa; Akazawa, Kohei; Tanaka, Takahiro; Yagi, Keita; Young, Lisa R; McCormack, Francis X; Nakata, Koh 2016. Efficacy and Safety of Long-term Sirolimus Therapy for Asian Patients with Lymphangioleiomyomatosis. Annals of the American Thoracic Society, , Goldberg, Hilary J; Harari, Sergio; Cottin, Vincent; Rosas, Ivan O; Peters, Elizabeth; Biswal, Shibadas; Cheng, Yi; Khindri, Sanjeev; Kovarik, John M; Ma, Shenglin; McCormack, Francis X; Henske, Elizabeth Petri 2015. Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study. The European respiratory journal, 46 3, 783-94Gupta, Nishant; Han, MeiLan K; McCormack, Francis X 2015. Regional Sparing in an Oligemic Lung Segment Supports Hematogenous Spread as a Pathogenic Mechanism in Lymphangioleiomyomatosis. Annals of the American Thoracic Society, 12 8, 1247-8Gupta, Nishant; Meraj, Riffat; Tanase, Daniel; James, Laura E; Seyama, Kuniaki; Lynch, David A; Akira, Masanori; Meyer, Cristopher A; Ruoss, Stephen J; Burger, Charles D; Young, Lisa R; Almoosa, Khalid F; Veeraraghavan, Srihari; Barker, Alan F; Lee, Augustine S; Dilling, Daniel F; Inoue, Yoshikazu; Cudzilo, Corey J; Zafar, Muhammad A; McCormack, Francis X 2015. Accuracy of chest high-resolution computed tomography in diagnosing diffuse cystic lung diseases. The European respiratory journal, 46 4, 1196-9Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2015. Diffuse Cystic Lung Disease. Part II. American journal of respiratory and critical care medicine, 192 1, 17-29Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X 2015. Diffuse Cystic Lung Disease. Part I. American journal of respiratory and critical care medicine, 191 12, 1354-66Gupta, Rajeev; McCormack, Francis X 2015. Response. Lymphology, 48 1, 52Mulhall, Aaron M; McCormack, Francis X 2015. A 25-year-old man with fatigue, dyspnea, and pulmonary cysts. Annals of the American Thoracic Society, 12 1, 120-3Pasula, Rajamouli; Azad, Abul K; Gardner, Jason C; Schlesinger, Larry S; McCormack, Francis X 2015. Keratinocyte growth factor administration attenuates murine pulmonary mycobacterium tuberculosis infection through granulocyte-macrophage colony-stimulating factor (GM-CSF)-dependent macrophage activation and phagolysosome fusion. The Journal of biological chemistry, 290 11, 7151-9Quintanilla, Hector D; Liu, Yuying; Fatheree, Nicole Y; Atkins, Constance L; Hashmi, Syed S; Floros, Joanna; McCormack, Francis X; Rhoads, Jon Marc; Alcorn, Joseph L 2015. Oral administration of surfactant protein-a reduces pathology in an experimental model of necrotizing enterocolitis. Journal of pediatric gastroenterology and nutrition, 60 5, 613-20Saito, Atsushi; Nikolaidis, Nikolaos M; Amlal, Hassane; Uehara, Yasuaki; Gardner, Jason C; LaSance, Kathleen; Pitstick, Lori B; Bridges, James P; Wikenheiser-Brokamp, Kathryn A; McGraw, Dennis W; Woods, Jason C; Sabbagh, Yves; Schiavi, Susan C; Altini?ik, Göksel; Jakopovi?, Marko; Inoue, Yoshikazu; McCormack, Francis X 2015. Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment. Science translational medicine, 7 313, 313ra181Yang, Linlin; Carrillo, Marykate; Wu, Yuchieh M; DiAngelo, Susan L; Silveyra, Patricia; Umstead, Todd M; Halstead, E Scott; Davies, Michael L; Hu, Sanmei; Floros, Joanna; McCormack, Francis X; Christensen, Neil D; Chroneos, Zissis C 2015. SP-R210 (Myo18A) Isoforms as Intrinsic Modulators of Macrophage Priming and Activation. PloS one, 10 5, e0126576Cundiff, William B; McCormack, Francis X; Wikenheiser-Brokamp, Kathryn; Starnes, Sandra; Kotloff, Robert; Benzaquen, Sadia 2014. Successful management of a chronic, refractory bronchopleural fistula with endobronchial valves followed by talc pleurodesis. American journal of respiratory and critical care medicine, 189 4, 490-1Gardner, Jason C; Noel, John G; Nikolaidis, Nikolaos M; Karns, Rebekah; Aronow, Bruce J; Ogle, Cora K; McCormack, Francis X 2014. G-CSF drives a posttraumatic immune program that protects the host from infection. Journal of immunology (Baltimore, Md. : 1950), 192 5, 2405-17Gupta, R; Kitaichi, M; Inoue, Y; Kotloff, R; McCormack, F X 2014. Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology, 47 3, 106-17Gupta, R; Kitaichi, M; Inoue, Y; Kotloff, R; McCormack, F X 2014. Lymphatic manifestations of lymphangioleiomyomatosis. Lymphology, 47 3, 106-17McCormack, Francis X 2014. Chronic sirolimus therapy for lymphangioleiomyomatosis. American journal of respiratory and critical care medicine, 190 12, 1332-3Nikolaidis, Nikolaos M; White, Mitchell R; Allen, Kimberly; Tripathi, Shweta; Qi, Li; McDonald, Barbara; Taubenberger, Jeffery; Seaton, Barbara A; McCormack, Francis X; Crouch, Erika C; Hartshorn, Kevan L 2014. Mutations flanking the carbohydrate binding site of surfactant protein D confer antiviral activity for pandemic influenza A viruses. American journal of physiology. Lung cellular and molecular physiology, 306 11, L1036-44Safi, Fadi; Gupta, Anita; Adams, Denise; Anandan, Vasuki; McCormack, Francis X; Assaly, Ragheb 2014. Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. Annals of the American Thoracic Society, 11 1, 92-5Cudzilo, Corey J; Szczesniak, Rhonda D; Brody, Alan S; Rattan, Mantosh S; Krueger, Darcy A; Bissler, John J; Franz, David N; McCormack, Francis X; Young, Lisa R 2013. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest, 144 2, 578-85Czyzyk-Krzeska, Maria F; McCormack, Francis X 2013. Birt-Hogg-Dubé syndrome. Familial cancer, 12 3, 355-6Gupta, Nishant; McCormack, Francis X 2013. Pulmonary alveolar microlithiasis. American journal of respiratory and critical care medicine, 188 7, e11-2Gupta, Nishant; Seyama, Kuniaki; McCormack, Francis X 2013. Pulmonary manifestations of Birt-Hogg-Dubé syndrome. Familial cancer, 12 3, 387-96Krueger, Darcy A; Northrup, Hope 2013. Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatric neurology, 49 4, 255-65McCormack, Francis X; Travis, William D; Colby, Thomas V; Henske, Elizabeth P; Moss, Joel 2013. Reply: Characterization of lymphangioleiomyomatosis as a neoplastic disease. American journal of respiratory and critical care medicine, 188 3, 398-9McCormack, Francis X; Wilmott, Clay; Young, Lisa R; Wikenheiser-Brokamp, Kathryn 2013. An atoll variant that mocks. American journal of respiratory and critical care medicine, 188 1, 110Northrup, Hope; Krueger, Darcy A 2013. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 Iinternational Tuberous Sclerosis Complex Consensus Conference. Pediatric neurology, 49 4, 243-54Oprescu, N; McCormack, F X; Byrnes, S; Kinder, B W 2013. Clinical predictors of mortality and cause of death in lymphangioleiomyomatosis: a population-based registry. Lung, 191 1, 35-42Safi, Fadi; Gupta, Anita; Adams, Denise; Anandan, Vasuki; McCormack, Francis X; Assaly, Ragheb 2013. Kaposiform Lymphangiomatosis, a Newly Characterized Vascular Anomaly Presenting with Hemoptysis in an Adult Woman. Annals of the American Thoracic Society, , Swigris, Jeffrey J; Lee, Hye-Seung; Cohen, Marsha; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Young, Lisa R; McCormack, Francis X 2013. St. George's Respiratory Questionnaire has longitudinal construct validity in lymphangioleiomyomatosis. Chest, 143 6, 1671-8Young, Lisa R; Lee, Hye-Seung; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Strange, Charlie; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Downey, Gregory P; Swigris, Jeffrey J; Taveira-Dasilva, Angelo M; Krischer, Jeffrey P; Trapnell, Bruce C; McCormack, Francis X 2013. Serum VEGF-D concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. The lancet. Respiratory medicine, 1 6, 445-452Zafar, Muhammad A; McCormack, Francis X; Rahman, Shahid; Tencza, Christian; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; Shizukuda, Yukitaka; Elwing, Jean M 2013. Pulmonary vascular shunts in exercise-intolerant patients with lymphangioleiomyomatosis. American journal of respiratory and critical care medicine, 188 9, 1167-70Henske, Elizabeth P; McCormack, Francis X 2012. Lymphangioleiomyomatosis - a wolf in sheep's clothing. The Journal of clinical investigation, 122 11, 3807-16McCormack, Francis X; Travis, William D; Colby, Thomas V; Henske, Elizabeth P; Moss, Joel 2012. Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. American journal of respiratory and critical care medicine, 186 12, 1210-2Meraj, Riffat; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; Byrnes, Sue; McCormack, Francis X 2012. Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis. Frontiers of medicine, 6 4, 395-405Meraj, Riffat; Wikenheiser-Brokamp, Kathryn A; Young, Lisa R; McCormack, Francis X 2012. Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Seminars in respiratory and critical care medicine, 33 5, 486-97Young, Lisa R; Gulleman, Peter M; Bridges, James P; Weaver, Timothy E; Deutsch, Gail H; Blackwell, Timothy S; McCormack, Francis X 2012. The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome. American journal of respiratory and critical care medicine, 186 10, 1014-24Crouch, Erika; Nikolaidis, Nikolaos; McCormack, Francis X; McDonald, Barbara; Allen, Kimberly; Rynkiewicz, Michael J; Cafarella, Tanya M; White, Mitchell; Lewnard, Kara; Leymarie, Nancy; Zaia, Joseph; Seaton, Barbara A; Hartshorn, Kevan L 2011. Mutagenesis of surfactant protein D informed by evolution and x-ray crystallography enhances defenses against influenza A virus in vivo. The Journal of biological chemistry, 286 47, 40681-92Hagaman, Jared T; Panos, Ralph J; McCormack, Francis X; Thakar, Charuhas V; Wikenheiser-Brokamp, Kathryn A; Shipley, Ralph T; Kinder, Brent W 2011. Vitamin D deficiency and reduced lung function in connective tissue-associated interstitial lung diseases. Chest, 139 2, 353-60McCormack, Francis X; Inoue, Yoshikazu; Moss, Joel; Singer, Lianne G; Strange, Charlie; Nakata, Koh; Barker, Alan F; Chapman, Jeffrey T; Brantly, Mark L; Stocks, James M; Brown, Kevin K; Lynch, Joseph P; Goldberg, Hilary J; Young, Lisa R; Kinder, Brent W; Downey, Gregory P; Sullivan, Eugene J; Colby, Thomas V; McKay, Roy T; Cohen, Marsha M; Korbee, Leslie; Taveira-DaSilva, Angelo M; Lee, Hye-Seung; Krischer, Jeffrey P; Trapnell, Bruce C 2011. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. The New England journal of medicine, 364 17, 1595-606Seaman, Danielle M; Meyer, Cristopher A; Gilman, Matthew D; McCormack, Francis X 2011. Diffuse Cystic Lung Disease at High-Resolution CT. AJR. American journal of roentgenology, 196 6, 1305-11Shang, Feifei; Rynkiewicz, Michael J; McCormack, Francis X; Wu, Huixing; Cafarella, Tanya M; Head, James F; Seaton, Barbara A 2011. Crystallographic complexes of surfactant protein A and carbohydrates reveal ligand-induced conformational change. The Journal of biological chemistry, 286 1, 757-65Wu, Huixing; Suzuki, Takuji; Carey, Brenna; Trapnell, Bruce C; McCormack, Francis X 2011. Keratinocyte growth factor augments pulmonary innate immunity through epithelium-driven, GM-CSF-dependent paracrine activation of alveolar macrophages. The Journal of biological chemistry, 286 17, 14932-40Franz, D N; Bissler, J J; McCormack, F X 2010. Tuberous sclerosis complex: neurological, renal and pulmonary manifestations. Neuropediatrics, 41 5, 199-208Hagaman, Jared T; Ferguson, Bryce; Meyer, Cris; Wang, Jiang; McCormack, Francis X; Nath, Amaresh 2010. Endobronchial Carcinoid Tumor in an HIV-infected Patient. Journal of bronchology & interventional pulmonology, 17 2, 174-6Hagaman, Jared T; Schauer, Daniel P; McCormack, Francis X; Kinder, Brent W 2010. Screening for lymphangioleiomyomatosis by high-resolution computed tomography in young, nonsmoking women presenting with spontaneous pneumothorax is cost-effective. American journal of respiratory and critical care medicine, 181 12, 1376-82Johnson, S R; Cordier, J F; Lazor, R; Cottin, V; Costabel, U; Harari, S; Reynaud-Gaubert, M; Boehler, A; Brauner, M; Popper, H; Bonetti, F; Kingswood, C 2010. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. The European respiratory journal, 35 1, 14-26Kinder, Brent W; Sherman, A C; Young, L R; Hagaman, J T; Oprescu, N; Byrnes, S; McCormack, Francis X 2010. Predictors for clinical trial participation in the rare lung disease lymphangioleiomyomatosis. Respiratory medicine, 104 4, 578-83Kinder, Brent; McCormack, Francis X 2010. Clinical trials for rare lung diseases: lessons from lymphangioleiomyomatosis. Lymphatic research and biology, 8 1, 71-9Seaton, Barbara A; Crouch, Erika C; McCormack, Francis X; Head, James F; Hartshorn, Kevan L; Mendelsohn, Richard 2010. Review: Structural determinants of pattern recognition by lung collectins. Innate immunity, 16 3, 143-50Young, Lisa R; Vandyke, Rhonda; Gulleman, Peter M; Inoue, Yoshikazu; Brown, Kevin K; Schmidt, Laura S; Linehan, W Marston; Hajjar, Fuad; Kinder, Brent W; Trapnell, Bruce C; Bissler, John J; Franz, David N; McCormack, Francis X 2010. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest, 138 3, 674-81Gil, Malgorzata; McCormack, Francis X; Levine, Ann Marie 2009. Surfactant protein A modulates cell surface expression of CR3 on alveolar macrophages and enhances CR3-mediated phagocytosis. The Journal of biological chemistry, 284 12, 7495-504Kinder, Brent W; Brown, Kevin K; McCormack, Francis X; Ix, Joachim H; Kervitsky, Alma; Schwarz, Marvin I; King, Talmadge E 2009. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest, 135 6, 1557-63Schmithorst, Vincent J; Altes, Talissa A; Young, Lisa R; Franz, David N; Bissler, John J; McCormack, Francis X; Dardzinski, Bernard J; Brody, Alan S 2009. Automated algorithm for quantifying the extent of cystic change on volumetric chest CT: initial results in Lymphangioleiomyomatosis. AJR. American journal of roentgenology, 192 4, 1037-44Young, Lisa R; Franz, David N; Nagarkatte, Preeti; Fletcher, Christopher D M; Wikenheiser-Brokamp, Kathryn A; Galsky, Matthew D; Corbridge, Thomas C; Lam, Anna P; Gelfand, Michael J; McCormack, Francis X 2009. Utility of [18F]2-fluoro-2-deoxyglucose-PET in sporadic and tuberous sclerosis-associated lymphangioleiomyomatosis. Chest, 136 3, 926-33Ali, Naeem A; O'Brien, James M; Hoffmann, Stephen P; Phillips, Gary; Garland, Allan; Finley, James C W; Almoosa, Khalid; Hejal, Rana; Wolf, Karen M; Lemeshow, Stanley; Connors, Alfred F; Marsh, Clay B 2008. Acquired weakness, handgrip strength, and mortality in critically ill patients. American journal of respiratory and critical care medicine, 178 3, 261-8Bissler, John J; McCormack, Francis X; Young, Lisa R; Elwing, Jean M; Chuck, Gail; Leonard, Jennifer M; Schmithorst, Vincent J; Laor, Tal; Brody, Alan S; Bean, Judy; Salisbury, Shelia; Franz, David N 2008. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. The New England journal of medicine, 358 2, 140-51McCormack, Francis X 2008. Lymphangioleiomyomatosis: a clinical update. Chest, 133 2, 507-16Young, Lisa R; Inoue, Yoshikazu; McCormack, Francis X 2008. Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis. The New England journal of medicine, 358 2, 199-200Byrnes, Abigail B; McCormack, Francis X; Diers, Tiffiny; Jazieh, Abdul-Rahman 2007. The resident scholar program: a research training opportunity for internal medicine house staff. Journal of cancer education : the official journal of the American Association for Cancer Education, 22 1, 47-9Juvet, Stephen C; McCormack, Francis X; Kwiatkowski, David J; Downey, Gregory P 2007. Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphans. American journal of respiratory cell and molecular biology, 36 4, 398-408McCormack, Francis X; Moss, Joel 2007. S-LAM in a man? American journal of respiratory and critical care medicine, 176 1, 3-5Pollock-BarZiv, Stacey; Cohen, Marsha M; Downey, Gregory P; Johnson, Simon R; Sullivan, Eugene; McCormack, Francis X 2007. Air travel in women with lymphangioleiomyomatosis. Thorax, 62 2, 176-80Young, Lisa R; Pasula, Rajamouli; Gulleman, Peter M; Deutsch, Gail H; McCormack, Francis X 2007. Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosis. American journal of respiratory cell and molecular biology, 37 1, 67-74Zhang, Shiping; McCormack, Francis X; Levesque, Roger C; O'Toole, George A; Lau, Gee W 2007. The flagellum of Pseudomonas aeruginosa is required for resistance to clearance by surfactant protein A. PloS one, 2 6, e564Almoosa, Khalid F; McCormack, Francis X; Sahn, Steven A 2006. Pleural disease in lymphangioleiomyomatosis. Clinics in chest medicine, 27 2, 355-68Almoosa, Khalid F; Ryu, Jay H; Mendez, Jose; Huggins, J Terrill; Young, Lisa R; Sullivan, Eugene J; Maurer, Janet; McCormack, Francis X; Sahn, Steven A 2006. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest, 129 5, 1274-81Kingma, Paul S; Zhang, Liqian; Ikegami, Machiko; Hartshorn, Kevan; McCormack, Francis X; Whitsett, Jeffrey A 2006. Correction of pulmonary abnormalities in Sftpd-/- mice requires the collagenous domain of surfactant protein D. The Journal of biological chemistry, 281 34, 24496-505Kuzmenko, Alexander I; Wu, Huixing; McCormack, Francis X 2006. Pulmonary collectins selectively permeabilize model bacterial membranes containing rough lipopolysaccharide. Biochemistry, 45 8, 2679-85McCormack, Francis X 2006. Lymphangioleiomyomatosis. MedGenMed : Medscape general medicine, 8 1, 15McCormack, Francis X 2006. New concepts in collectin-mediated host defense at the air-liquid interface of the lung. Respirology (Carlton, Vic.), 11 Suppl , S7-10Ryan, Marnie A; Akinbi, Henry T; Serrano, Alicia G; Perez-Gil, Jesus; Wu, Huixing; McCormack, Francis X; Weaver, Timothy E 2006. Antimicrobial activity of native and synthetic surfactant protein B peptides. Journal of immunology (Baltimore, Md. : 1950), 176 1, 416-25Ryu, Jay H; Moss, Joel; Beck, Gerald J; Lee, Jar-Chi; Brown, Kevin K; Chapman, Jeffrey T; Finlay, Geraldine A; Olson, Eric J; Ruoss, Stephen J; Maurer, Janet R; Raffin, Thomas A; Peavy, Hannah H; McCarthy, Kevin; Taveira-Dasilva, Angelo; McCormack, Francis X; Avila, Nilo A; Decastro, Rosamma M; Jacobs, Susan S; Stylianou, Mario; Fanburg, Barry L 2006. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. American journal of respiratory and critical care medicine, 173 1, 105-11Schlosser, A; Thomsen, T; Shipley, J M; Hein, P W; Brasch, F; Tornøe, I; Nielsen, O; Skjødt, K; Palaniyar, N; Steinhilber, W; McCormack, F X; Holmskov, U 2006. Microfibril-associated protein 4 binds to surfactant protein A (SP-A) and colocalizes with SP-A in the extracellular matrix of the lung. Scandinavian journal of immunology, 64 2, 104-16Young, Lisa R; Almoosa, Khalid F; Pollock-Barziv, Stacey; Coutinho, Meg; McCormack, Francis X; Sahn, Steven A 2006. Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis. Chest, 129 5, 1267-73Young, Lisa R; Borchers, Michael T; Allen, Holly L; Gibbons, Reta S; McCormack, Francis X 2006. Lung-restricted macrophage activation in the pearl mouse model of Hermansky-Pudlak syndrome. Journal of immunology (Baltimore, Md. : 1950), 176 7, 4361-8Zhang, Liqian; Ikegami, Machiko; Korfhagen, Thomas R; McCormack, Francis X; Yoshida, Mitsuhiro; Senior, Robert M; Shipley, J Michael; Shapiro, Steven D; Whitsett, Jeffrey A 2006. Neither SP-A nor NH2-terminal domains of SP-A can substitute for SP-D in regulation of alveolar homeostasis. American journal of physiology. Lung cellular and molecular physiology, 291 2, L181-90Beharka, Alison A; Crowther, Joy E; McCormack, Francis X; Denning, Gerene M; Lees, Jason; Tibesar, Eric; Schlesinger, Larry S 2005. Pulmonary surfactant protein A activates a phosphatidylinositol 3-kinase/calcium signal transduction pathway in human macrophages: participation in the up-regulation of mannose receptor activity. Journal of immunology (Baltimore, Md. : 1950), 175 4, 2227-36Kuzmenko, Alexander I; Wu, Huixing; Wan, Sijue; McCormack, Francis X 2005. Surfactant protein A is a principal and oxidation-sensitive microbial permeabilizing factor in the alveolar lining fluid. The Journal of biological chemistry, 280 27, 25913-9Zhang, Shiping; Chen, Yi; Potvin, Eric; Sanschagrin, Francois; Levesque, Roger C; McCormack, Francis X; Lau, Gee W 2005. Comparative signature-tagged mutagenesis identifies Pseudomonas factors conferring resistance to the pulmonary collectin SP-A. PLoS pathogens, 1 3, 259-68Crowther, Joy E; Kutala, Vijay Kumar; Kuppusamy, Periannan; Ferguson, J Scott; Beharka, Alison A; Zweier, Jay L; McCormack, Francis X; Schlesinger, Larry S 2004. Pulmonary surfactant protein a inhibits macrophage reactive oxygen intermediate production in response to stimuli by reducing NADPH oxidase activity. Journal of immunology (Baltimore, Md. : 1950), 172 11, 6866-74Kuzmenko, A I; Wu, H; Bridges, J P; McCormack, F X 2004. Surfactant lipid peroxidation damages surfactant protein A and inhibits interactions with phospholipid vesicles. Journal of lipid research, 45 6, 1061-8Schaeffer, Lyndsay M; McCormack, Francis X; Wu, Huixing; Weiss, Alison A 2004. Interactions of pulmonary collectins with Bordetella bronchiseptica and Bordetella pertussis lipopolysaccharide elucidate the structural basis of their antimicrobial activities. Infection and immunity, 72 12, 7124-30Schaeffer, Lyndsay M; McCormack, Francis X; Wu, Huixing; Weiss, Alison A 2004. Bordetella pertussis lipopolysaccharide resists the bactericidal effects of pulmonary surfactant protein A. Journal of immunology (Baltimore, Md. : 1950), 173 3, 1959-65Chabot, Sophie; Salez, Laurent; McCormack, Francis X; Touqui, Lhousseine; Chignard, Michel 2003. Surfactant protein A inhibits lipopolysaccharide-induced in vivo production of interleukin-10 by mononuclear phagocytes during lung inflammation. American journal of respiratory cell and molecular biology, 28 3, 347-53Head, James F; Mealy, Tanya R; McCormack, Francis X; Seaton, Barbara A 2003. Crystal structure of trimeric carbohydrate recognition and neck domains of surfactant protein A. The Journal of biological chemistry, 278 44, 43254-60Karbowniczek, Magdalena; Astrinidis, Aristotelis; Balsara, Binaifer R; Testa, Joseph R; Lium, James H; Colby, Thomas V; McCormack, Francis X; Henske, Elizabeth Petri 2003. Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism. American journal of respiratory and critical care medicine, 167 7, 976-82McCormack, Francis X; Gibbons, Reta; Ward, Susan R; Kuzmenko, Alexander; Wu, Huixing; Deepe, George S 2003. Macrophage-independent fungicidal action of the pulmonary collectins. The Journal of biological chemistry, 278 38, 36250-6Rodriguez Capote, Karina; McCormack, Francis X; Possmayer, Fred 2003. Pulmonary surfactant protein-A (SP-A) restores the surface properties of surfactant after oxidation by a mechanism that requires the Cys6 interchain disulfide bond and the phospholipid binding domain. The Journal of biological chemistry, 278 23, 20461-74Sietsema, Kathy E; McCormack, Francis X 2003. Lymphangioleiomyomatosis: insights about, and from, a rare disease. American journal of respiratory and critical care medicine, 168 12, 1405-6Wu, Huixing; Kuzmenko, Alexander; Wan, Sijue; Schaffer, Lyndsay; Weiss, Alison; Fisher, James H; Kim, Kwang Sik; McCormack, Francis X 2003. Surfactant proteins A and D inhibit the growth of Gram-negative bacteria by increasing membrane permeability. The Journal of clinical investigation, 111 10, 1589-602Beharka, Alison A; Gaynor, Cecilia D; Kang, Byoung K; Voelker, Dennis R; McCormack, Francis X; Schlesinger, Larry S 2002. Pulmonary surfactant protein A up-regulates activity of the mannose receptor, a pattern recognition receptor expressed on human macrophages. Journal of immunology (Baltimore, Md. : 1950), 169 7, 3565-73Guillot, Loïc; Balloy, Viviane; McCormack, Francis X; Golenbock, Douglas T; Chignard, Michel; Si-Tahar, Mustapha 2002. Cutting edge: the immunostimulatory activity of the lung surfactant protein-A involves Toll-like receptor 4. Journal of immunology (Baltimore, Md. : 1950), 168 12, 5989-92Malloy, Jaret L; Veldhuizen, Ruud A W; McCormack, Francis X; Korfhagen, Thomas R; Whitsett, Jeffery A; Lewis, James F 2002. Pulmonary surfactant and inflammation in septic adult mice: role of surfactant protein A. Journal of applied physiology (Bethesda, Md. : 1985), 92 2, 809-16McCormack, Francis X; Whitsett, Jeffrey A 2002. The pulmonary collectins, SP-A and SP-D, orchestrate innate immunity in the lung. The Journal of clinical investigation, 109 6, 707-12McCormack, Francis; Brody, Alan; Meyer, Christopher; Leonard, Jennifer; Chuck, Gail; Dabora, Sandra; Sethuraman, Gopalan; Colby, Thomas V; Kwiatkowski, David J; Franz, David N 2002. Pulmonary cysts consistent with lymphangioleiomyomatosis are common in women with tuberous sclerosis: genetic and radiographic analysis. Chest, 121 3 Suppl, 61SPalaniyar, Nades; Zhang, Liquian; Kuzmenko, Alexander; Ikegami, Machiko; Wan, Sijue; Wu, Huixing; Korfhagen, Thomas R; Whitsett, Jeffrey A; McCormack, Francis X 2002. The role of pulmonary collectin N-terminal domains in surfactant structure, function, and homeostasis in vivo. The Journal of biological chemistry, 277 30, 26971-9Franz, D N; Brody, A; Meyer, C; Leonard, J; Chuck, G; Dabora, S; Sethuraman, G; Colby, T V; Kwiatkowski, D J; McCormack, F X 2001. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. American journal of respiratory and critical care medicine, 164 4, 661-8Ikegami, M; Elhalwagi, B M; Palaniyar, N; Dienger, K; Korfhagen, T; Whitsett, J A; McCormack, F X 2001. The collagen-like region of surfactant protein A (SP-A) is required for correction of surfactant structural and functional defects in the SP-A null mouse. The Journal of biological chemistry, 276 42, 38542-8Linke, M J; Harris, C E; Korfhagen, T R; McCormack, F X; Ashbaugh, A D; Steele, P; Whitsett, J A; Walzer, P D 2001. Immunosuppressed surfactant protein A-deficient mice have increased susceptibility to Pneumocystis carinii infection. The Journal of infectious diseases, 183 6, 943-52Palaniyar, N; Ikegami, M; Korfhagen, T; Whitsett, J; McCormack, F X 2001. Domains of surfactant protein A that affect protein oligomerization, lipid structure and surface tension. Comparative biochemistry and physiology. Part A, Molecular & integrative physiology, 129 1, 109-27Salez, L; Balloy, V; van Rooijen, N; Lebastard, M; Touqui, L; McCormack, F X; Chignard, M 2001. Surfactant protein A suppresses lipopolysaccharide-induced IL-10 production by murine macrophages. Journal of immunology (Baltimore, Md. : 1950), 166 10, 6376-82Bridges, J P; Davis, H W; Damodarasamy, M; Kuroki, Y; Howles, G; Hui, D Y; McCormack, F X 2000. Pulmonary surfactant proteins A and D are potent endogenous inhibitors of lipid peroxidation and oxidative cellular injury. The Journal of biological chemistry, 275 49, 38848-55Damodarasamy, M; Zhang, M; Dienger, K; McCormack, F X 2000. Two rat surfactant protein A isoforms arise by a novel mechanism that includes alternative translation initiation. Biochemistry, 39 33, 10189-95Fisher, J H; Sheftelyevich, V; Ho, Y S; Fligiel, S; McCormack, F X; Korfhagen, T R; Whitsett, J A; Ikegami, M 2000. Pulmonary-specific expression of SP-D corrects pulmonary lipid accumulation in SP-D gene-targeted mice. American journal of physiology. Lung cellular and molecular physiology, 278 2, L365-73Palaniyar, N; McCormack, F X; Possmayer, F; Harauz, G 2000. Three-dimensional structure of rat surfactant protein A trimers in association with phospholipid monolayers. Biochemistry, 39 21, 6310-6Elhalwagi, B M; Zhang, M; Ikegami, M; Iwamoto, H S; Morris, R E; Miller, M L; Dienger, K; McCormack, F X 1999. Normal surfactant pool sizes and inhibition-resistant surfactant from mice that overexpress surfactant protein A. American journal of respiratory cell and molecular biology, 21 3, 380-7Ferguson, J S; Voelker, D R; McCormack, F X; Schlesinger, L S 1999. Surfactant protein D binds to Mycobacterium tuberculosis bacilli and lipoarabinomannan via carbohydrate-lectin interactions resulting in reduced phagocytosis of the bacteria by macrophages. Journal of immunology (Baltimore, Md. : 1950), 163 1, 312-21McCormack, F X; Damodarasamy, M; Elhalwagi, B M 1999. Deletion mapping of N-terminal domains of surfactant protein A. The N-terminal segment is required for phospholipid aggregation and specific inhibition of surfactant secretion. The Journal of biological chemistry, 274 5, 3173-81Yu, S H; McCormack, F X; Voelker, D R; Possmayer, F 1999. Interactions of pulmonary surfactant protein SP-A with monolayers of dipalmitoylphosphatidylcholine and cholesterol: roles of SP-A domains. Journal of lipid research, 40 5, 920-9Borron, P; McCormack, F X; Elhalwagi, B M; Chroneos, Z C; Lewis, J F; Zhu, S; Wright, J R; Shepherd, V L; Possmayer, F; Inchley, K; Fraher, L J 1998. Surfactant protein A inhibits T cell proliferation via its collagen-like tail and a 210-kDa receptor. The American journal of physiology, 275 4 Pt 1, L679-86McCormack, F X 1998. Structure, processing and properties of surfactant protein A. Biochimica et biophysica acta, 1408 2-3, 109-31Pattanajitvilai, S; Kuroki, Y; Tsunezawa, W; McCormack, F X; Voelker, D R 1998. Mutational analysis of Arg197 of rat surfactant protein A. His197 creates specific lipid uptake defects. The Journal of biological chemistry, 273 10, 5702-7Smolarek, T A; Wessner, L L; McCormack, F X; Mylet, J C; Menon, A G; Henske, E P 1998. Evidence that lymphangiomyomatosis is caused by TSC2 mutations: chromosome 16p13 loss of heterozygosity in angiomyolipomas and lymph nodes from women with lymphangiomyomatosis. American journal of human genetics, 62 4, 810-5Sunkin, S M; Linke, M J; McCormack, F X; Walzer, P D; Stringer, J R 1998. Identification of a putative precursor to the major surface glycoprotein of Pneumocystis carinii. Infection and immunity, 66 2, 741-6Tsunezawa, W; Sano, H; Sohma, H; McCormack, F X; Voelker, D R; Kuroki, Y 1998. Site-directed mutagenesis of surfactant protein A reveals dissociation of lipid aggregation and lipid uptake by alveolar type II cells. Biochimica et biophysica acta, 1387 1-2, 433-46Zhang, M; Damodarasamy, M; Elhalwagi, B M; McCormack, F X 1998. The longer isoform and Cys-1 disulfide bridge of rat surfactant protein A are not essential for phospholipid and type II cell interactions. Biochemistry, 37 47, 16481-8Blau, H; Riklis, S; Van Iwaarden, J F; McCormack, F X; Kalina, M 1997. Nitric oxide production by rat alveolar macrophages can be modulated in vitro by surfactant protein A. The American journal of physiology, 272 6 Pt 1, L1198-204Elhalwagi, B M; Damodarasamy, M; McCormack, F X 1997. Alternate amino terminal processing of surfactant protein A results in cysteinyl isoforms required for multimer formation. Biochemistry, 36 23, 7018-25McCormack, F 1997. The structure and function of surfactant protein-A. Chest, 111 6 Suppl, 114S-119SMcCormack, F X; Festa, A L; Andrews, R P; Linke, M; Walzer, P D 1997. The carbohydrate recognition domain of surfactant protein A mediates binding to the major surface glycoprotein of Pneumocystis carinii. Biochemistry, 36 26, 8092-9McCormack, F X; Pattanajitvilai, S; Stewart, J; Possmayer, F; Inchley, K; Voelker, D R 1997. The Cys6 intermolecular disulfide bond and the collagen-like region of rat SP-A play critical roles in interactions with alveolar type II cells and surfactant lipids. The Journal of biological chemistry, 272 44, 27971-9McCormack, F X; Stewart, J; Voelker, D R; Damodarasamy, M 1997. Alanine mutagenesis of surfactant protein A reveals that lipid binding and pH-dependent liposome aggregation are mediated by the carbohydrate recognition domain. Biochemistry, 36 45, 13963-71Gaynor, C D; McCormack, F X; Voelker, D R; McGowan, S E; Schlesinger, L S 1995. Pulmonary surfactant protein A mediates enhanced phagocytosis of Mycobacterium tuberculosis by a direct interaction with human macrophages. Journal of immunology (Baltimore, Md. : 1950), 155 11, 5343-51McCormack, F X; King, T E; Bucher, B L; Nielsen, L; Mason, R J; McCormac, F X 1995. Surfactant protein A predicts survival in idiopathic pulmonary fibrosis. American journal of respiratory and critical care medicine, 152 2, 751-9Kuroki, Y; McCormack, F X; Ogasawara, Y; Mason, R J; Voelker, D R 1994. Epitope mapping for monoclonal antibodies identifies functional domains of pulmonary surfactant protein A that interact with lipids. The Journal of biological chemistry, 269 47, 29793-800McCormack, F X; Calvert, H M; Watson, P A; Smith, D L; Mason, R J; Voelker, D R 1994. The structure and function of surfactant protein A. Hydroxyproline- and carbohydrate-deficient mutant proteins. The Journal of biological chemistry, 269 8, 5833-41McCormack, F X; Kuroki, Y; Stewart, J J; Mason, R J; Voelker, D R 1994. Surfactant protein A amino acids Glu195 and Arg197 are essential for receptor binding, phospholipid aggregation, regulation of secretion, and the facilitated uptake of phospholipid by type II cells. The Journal of biological chemistry, 269 47, 29801-7Ogasawara, Y; McCormack, F X; Mason, R J; Voelker, D R 1994. Chimeras of surfactant proteins A and D identify the carbohydrate recognition domains as essential for phospholipid interaction. The Journal of biological chemistry, 269 47, 29785-92de Carvalho, M S; McCormack, F X; Leslie, C C 1993. The 85-kDa, arachidonic acid-specific phospholipase A2 is expressed as an activated phosphoprotein in Sf9 cells. Archives of biochemistry and biophysics, 306 2, 534-40Kalina, M; McCormack, F X; Crowley, H; Voelker, D R; Mason, R J 1993. Internalization of surfactant protein A (SP-A) into lamellar bodies of rat alveolar type II cells in vitro. The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society, 41 1, 57-70Zimmerman, P E; Voelker, D R; McCormack, F X; Paulsrud, J R; Martin, W J 1992. 120-kD surface glycoprotein of Pneumocystis carinii is a ligand for surfactant protein A. The Journal of clinical investigation, 89 1, 143-9Fisher, J H; McCormack, F; Park, S S; Stelzner, T; Shannon, J M; Hofmann, T 1991. In vivo regulation of surfactant proteins by glucocorticoids. American journal of respiratory cell and molecular biology, 5 1, 63-70McCormack, F X; King, T E; Voelker, D R; Robinson, P C; Mason, R J 1991. Idiopathic pulmonary fibrosis. Abnormalities in the bronchoalveolar lavage content of surfactant protein A. The American review of respiratory disease, 144 1, 160-6McCormack, F X; Fisher, J H; Suwabe, A; Smith, D L; Shannon, J M; Voelker, D R 1990. Expression and characterization of rat surfactant protein A synthesized in Chinese hamster ovary cells. Biochimica et biophysica acta, 1087 2, 190-8Mackenzie, C F; Shin, B; Krishnaprasad, D; McCormack, F; Illingworth, W 1985. Assessment of cardiac and respiratory function during surgery on patients with acute quadriplegia. Journal of neurosurgery, 62 6, 843-9Sykes, J A; McCormack, F X; O'Brien, T J 1978. A preliminary study of the superoxide dismutase content of some human tumors. Cancer research, 38 9, 2759-62Kingma P.;Zhang L.;Ikegami M.;Hartshorn K.;McCormack F.;Whitsett J. 12-15-2006. Erratum: Correction of pulmonary abnormalities in Sftpd<sup>-/-</sup> mice requires the Journal of Biological Chemistry, 281 50, 38968Bridges J.P.;Davis H.W.;Damodarasamy M.;Kuroki Y.;Howles G.;Hui D.Y.;Francis-X. McCormack 12-13-2002. Erratum: Pulmonary surfactant proteins A and D are potent endogenous inhihitors of lipid peroxidatio Journal of Biological Chemistry, 277 50, 49090 White M.R.; Nikolaidis N.M.; McCormack F.; Crouch E.C.; Hartshorn K.L. 12-08-2021. Viral Evasion of Innate Immune Defense: The Case of Resistance of Pandemic H1N1 Influenza A Virus to Frontiers in Microbiology, 12 , Jose A.; Shah S.A.; Anwar N.; Jones C.R.; McCormack F.X.; Sherman K.E.; Elwing J.M. 12-01-2021. Predictors of outcomes following liver transplant in hepatopulmonary syndrome: An OPTN database anal Respiratory Medicine, 190 , Zhang S.;Chen Y.;Potvin E.;Sanschagrin F.;Levesque R.;McCormack F.;Lau G. 12-01-2005. Comparative signature-tagged mutagenesis identifies Pseudomonas factors conferring resistance to the PLoS Pathogens, 1 3, 0259-0268Blau H.;Riklis S.;Van Iwaarden J.;McCormack F.;Kalina M. 12-01-1997. Nitric oxide production by rat alveolar macrophages can be modulated in vitro by surfactant protein Pneumologie, 51 11, 1080Singla A.;Gupta N.;Apewokin S.;McCormack F. 11-02-2017. Sirolimus for the treatment of lymphangioleiomyomatosis Expert Opinion on Orphan Drugs, 5 11, 907-921 Northrup H.; Aronow M.E.; Bebin E.M.; Bissler J.; Darling T.N.; de Vries P.J.; Frost M.D.; Fuchs Z.; Gosnell E.S.; Gosnell E.S.; Gupta N.; Jansen A.C.; Jó?wiak S.; Kingswood J.C.; Knilans T.K.; McCormack F.X.; Pounders A.; Roberds S.L.; Rodriguez-Buritica D.F.; Rodriguez-Buritica D.F.; Roth J.; Sampson J.R.; Sparagana S.; Thiele E.A.; Weiner H.L.; Wheless J.W.; Towbin A.J.; Krueger D.A.; Annear N.M.P.; Bartels U.; Berhouma M.; Bissler J.J.; Budde K.; Byars A.; Chugani H.; Cowen E.W.; Crino P.B.; Curatolo P.; de Vries P.; Dilling D.F.; Dunn D.W.; Ekong R.; Ess K.C.; Franz D.N.; Frost M.; Fuchs Z.D.B.; Guay-Woodford L.; Haddad L.; Halbert A.; Hebert A.A.; Henske E.P.; Holmes G.L.; Hook D.; Hulbert J.; Jansen A.; Johnson S.R.; King B.; Kingswood J.C.; Koenig M.K.; Korf B.; Kwiatkowski D.J.; Moss J.; Mowat D.; Mowrey K.; Nabbout R.; Nellist M.D.; O'Callaghan F.; Patel U.; Roach E.S.; Romp R.; Rozenberg M.; Ruoss S.J.; Sahin M.; Sampson J.; Samuels J.A.; Sauter M.; Smith C.A.; Soltani K.; Srivastava S.; Stuart C.; Teng J.M.C.; Thiele E.A.; Trout A.; van Eeghen A.; Vanclooster S.; Wang H.Z.; Wataya-Kaneda M.; Witman P.; Wright T.; Wu J.Y.; Young L. 10-01-2021. Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Pediatric Neurology, 123 , 50-66 Zafar M.A.; Sengupta R.; Bates A.; Woods J.C.; Radchenko C.; McCormack F.X.; Panos R.J. 10-01-2021. Oral Positive Expiratory Pressure Device for Excessive Dynamic Airway Collapse Caused by Emphysema Chest, 160 4, e333-e337Kotloff R.;McCormack F. 09-01-2016. Rare Lung Diseases: Occasionally the “Horse” Has Stripes Clinics in Chest Medicine, 37 3, xv-xviMcCormack F.;Lee H.;Trapnell B. 07-21-2011. The authors reply New England Journal of Medicine, 365 3, 272McCormack F.;Henske E. 06-09-2010. Lymphangioleiomyomatosis and Pulmonary Disease in TSC Tuberous Sclerosis Complex: Genes, Clinical Features and Therapeutics, , 345-368Bissler J.;Young L.;McCormack F. 05-01-2008. Dr. Bissler and colleagues reply New England Journal of Medicine, 358 18, 1964McCormack F.X.;Inoue Y.;Moss J.;Singer L.G.;Strange C.;Nakata K.;Barker A.F.;Chapman J.T.;Brantly M.L.;Stocks J.M.;Brown K.K.;Lynch J.P.;Goldberg H.J.;Young L.R.;Kinder B.W.;Downey G.P.;Sullivan E.J.;Colby T.V.;McKay R.T.;Cohen M.M.;Korbee L.;Taveira-DaSilva A.M.;Lee H.S.;Krischer J.P.;Trapnell B.C. 04-28-2011. Efficacy and safety of sirolimus in lymphangioleiomyomatosis New England Journal of Medicine, 364 17, 1595-1606McCormack F. 04-19-2016. Lymphangioleiomyomatosis Interstitial Pulmonary and Bronchiolar Disorders, , 747-768 Warrior K.; Leard L.E.; Nair A.R.; Gries C.J.; Fisher A.J.; Johnson S.R.; McCormack F.X.; Dilling D.F. 04-01-2022. A survey of use of mTOR inhibitors in patients with lymphangioleiomyomatosis listed for lung transpl Respiratory Medicine, 195 , Struik M.H.L.;Van Moorsel C.H.M.;Van Beek F.T.;Veltkamp M.;Mccormack F.X.;Grutters J.C. 04-01-2015. The importance of biobank and nationwide registry for lymphangioleiomyomatosis in a small sized coun Expert Opinion on Orphan Drugs, 3 4, 393-401Hagaman J.T.;Ferguson B.;Meyer C.;Wang J.;McCormack F.X.;Nath A. 04-01-2010. Endobronchial carcinoid tumor in an HIV-infected patient Journal of Bronchology and Interventional Pulmonology, 17 2, 174-176McCormack F. 03-31-2006. Author's Reply to "Reader's response to 'Lymphangioleiomyomatosis'" MedGenMed Medscape General Medicine, 8 1, Sontake V.; Wang Y.; Kasam R.K.; Sinner D.; Reddy G.B.; Naren A.P.; McCormack F.X.; White E.S.; Jegga A.G.; Madala S.K. 02-23-2017. Hsp90 regulation of fibroblast activation in pulmonary fibrosis JCI Insight, 2 4, Lee L.H.; Gasilina A.; Roychoudhury J.; Clark J.; McCormack F.X.; Pressey J.; Grimley M.S.; Lorsbach R.; Ali S.; Bailey M.; Stephens P.; Ross J.S.; Miller V.A.; Nassar N.N.; Kumar A.R. 02-09-2017. Real-time genomic profiling of histiocytoses identifies early-kinase domain BRAF alterations while i JCI Insight, 2 3, Hagaman J.;Panos R.;McCormack F.;Thakar C.;Wikenheiser-Brokamp K.;Shipley R.;Kinder B. 02-01-2011. Vitamin D deficiency and reduced lung function in connective tissue-associated interstitial lung dis Chest, 139 2, 353-360 Du Y.; Guo M.; Wu Y.; Wagner A.; Perl A.K.; Wikenheiser-Brokamp K.; Yu J.; Gupta N.; Kopras E.; Krymskaya V.; Obraztsova K.; Tang Y.; Kwiatkowski D.; Henske E.P.; McCormack F.; Xu Y. 01-01-2022. Lymphangioleiomyomatosis (LAM) Cell Atlas Thorax, , Cudzilo C.J.;Szczesniak R.D.;Brody A.S.;Rattan M.S.;Krueger D.A.;Bissler J.J.;Franz D.N.;McCormack F.X.;Young L.R. 01-01-2013. Lymphangioleiomyomatosis screening in women with tuberous sclerosis Chest, 144 2, 578-585Swigris J.;Lee H.;Cohen M.;Inoue Y.;Moss J.;Singer L.;Young L.;McCormack F. 01-01-2013. St. George's respiratory questionnaire has longitudinal construct validity in lymphangioleiomyomatos Chest, 143 6, 1671-1678Johnson S.R.;Cordier J.F.;Lazor R.;Cottin V.;Costabel U.;Harari S.;Reynaud-Gaubert M.;Boehler A.;Brauner M.;Popper H.;Bonetti F.;Kingswood C.;Albera C.;Bissler J.;Bouros D.;Corris P.;Donnelly S.;Durand C.;Egan J.;Grutters J.C.;Hodgson U.;Hollis G.;Korzeniewska-Kosela M.;Kus J.;Lacronique J.;Lammers J.W.;McCormack F.;Mendes A.C.;Moss J.;Naalsund A.;Pohl W.;Radzikowska E.;Robalo-Cordeiro C.;Rouvière O.;Ryu J.;Schiavina M.;Tattersfield A.E.;Travis W.;Tukiainen P.;Urban T.;Valeyre D.;Verleden G.M. 01-01-2010. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis European Respiratory Journal, 35 1, 14-26McCormack F. 01-01-2008. Response Chest, 134 1, 218 McCormack F.X. 01-01-2005. Other rare lung diseases: Lymphangioleiomyomatosis Respiratory Genetics, , 479-489 Young L.R.; McCormack F.X. 01-01-2005. Other rare lung diseases: Hermansky-Pudlak Syndrome Respiratory Genetics, , 491-498Almoosa K.F.;Khoury S.F.;McCormack F.X. 01-01-2003. Acute myocardial infarction complicating sudden interruption of chronic prostacyclin therapy for pul Clinical Pulmonary Medicine, 10 4, 231-234Malloy J.;Veldhuizen R.;McCormack F.;Korfhagen T.;Whitsett J.;Lewis J. 01-01-2002. Pulmonary surfactant and inflammation in septic adult mice: Role of surfactant protein A Journal of Applied Physiology, 92 2, 809-816McCormack F.;King T.;Bucher B.;Nielsen L.;Mason R. 01-01-1995. Erratum: Surfactant protein A predicts survival in idiopathic pulmonary fibrosis (American Journal o American Journal of Respiratory and Critical Care Medicine, 152 4 I, 1425McCormack F.X. 01-01-1995. Molecular biology of the surfactant apoproteins Seminars in Respiratory and Critical Care Medicine, 16 1, 29-38De Carvalho M.;McCormack F.;Leslie C. 01-01-1993. The 85-kDa, Arachidonic Acid-Specific Phospholipase A<inf>2</inf> Is Expressed as an Act Archives of Biochemistry and Biophysics, 306 2, 534-540McCormack, F X . Functional mapping of surfactant protein A. Pediatric pathology & molecular medicine, 20 4, 293-318mccormack, f.x. . , , McCormack, Francis X . The way forward in lymphangioleiomyomatosis: a trial for every patient, every patient in a trial. Jornal brasileiro de pneumologia : publicaça?o oficial da Sociedade Brasileira de Pneumologia e Tisilogia, 37 4, 422-3