J
Joseph M. Pilewski
Researcher at University of Pittsburgh
Publications - 322
Citations - 17263
Joseph M. Pilewski is an academic researcher from University of Pittsburgh. The author has contributed to research in topics: Lung transplantation & Cystic fibrosis. The author has an hindex of 65, co-authored 285 publications receiving 15142 citations. Previous affiliations of Joseph M. Pilewski include Boston Children's Hospital & University of Pennsylvania.
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Journal ArticleDOI
IL-22 mediates mucosal host defense against Gram-negative bacterial pneumonia
Shean J. Aujla,Yvonne R. Chan,Mingquan Zheng,Mingjian Fei,David J. Askew,Derek Pociask,Todd A. Reinhart,Florencia McAllister,Jennifer Edeal,Kristi Gaus,Shahid Husain,James L. Kreindler,Patricia J. Dubin,Joseph M. Pilewski,Mike M. Myerburg,Carol A Mason,Yoichiro Iwakura,Jay K. Kolls +17 more
TL;DR: Although both cytokines regulated CXC chemokines and granulocyte colony–stimulating factor production in the lung, only IL-22 increased lung epithelial cell proliferation and increased transepithelial resistance to injury, and data support the concept that the TH17 cell lineage and its effector molecules have evolved to effect host defense against extracellular pathogens at mucosal sites.
Journal ArticleDOI
Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation
Frank J. Accurso,Steven M. Rowe,John P. Clancy,Michael P. Boyle,Jordan M. Dunitz,Peter R. Durie,Scott D. Sagel,Douglas B. Hornick,Michael W. Konstan,Scott H. Donaldson,Richard B. Moss,Joseph M. Pilewski,Ronald C. Rubenstein,Ahmet Uluer,Moira L. Aitken,Steven D. Freedman,Lynn M. Rose,Nicole Mayer-Hamblett,Qunming Dong,Jiuhong Zha,Anne Stone,Eric R. Olson,Claudia L. Ordoñez,Preston W. Campbell,Melissa A. Ashlock,Bonnie W. Ramsey +25 more
TL;DR: This study showed that VX-770 was associated with within-subject improvements in CFTR and lung function and provides support for further studies of pharmacologic potentiation of CFTR as a means to treat cystic fibrosis.
Journal ArticleDOI
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
John P. Clancy,Steven M. Rowe,Frank J. Accurso,Moira L. Aitken,Raouf S. Amin,Melissa A. Ashlock,Manfred Ballmann,Michael P. Boyle,Inez Bronsveld,Preston W. Campbell,Kris De Boeck,Scott H. Donaldson,Henry L. Dorkin,Jordan M. Dunitz,Peter R. Durie,Manu Jain,Anissa Leonard,Karen McCoy,Richard B. Moss,Joseph M. Pilewski,Daniel B. Rosenbluth,Ronald C. Rubenstein,Michael S. Schechter,Martyn Botfield,Claudia L. Ordoñez,George T. Spencer-Green,Laurent Vernillet,Steve Wisseh,Karl Yen,Michael W. Konstan +29 more
TL;DR: In this study, VX-809 had a similar adverse event profile to placebo for 28 days in F508del-CFTR homozygous patients, and demonstrated biological activity with positive impact on CFTR function in the sweat gland.
Journal ArticleDOI
Role of CFTR in airway disease
TL;DR: The details of this pathophysiological cascade, whereby the deletion or impairment of CFTR chloride channel function produces lung disease, are provided in the hope that its understanding will promote the development of new therapies for CF.
Journal ArticleDOI
Egr-1 Regulates Autophagy in Cigarette Smoke-Induced Chronic Obstructive Pulmonary Disease
Zhi Hua Chen,Hong Pyo Kim,Hong Pyo Kim,Frank C. Sciurba,Seon Jin Lee,Seon Jin Lee,Carol Feghali-Bostwick,Donna B. Stolz,Rajiv Dhir,Rodney J. Landreneau,Mathew J. Schuchert,Samuel A. Yousem,Kiichi Nakahira,Kiichi Nakahira,Joseph M. Pilewski,Janet S. Lee,Yingze Zhang,Stefan W. Ryter,Stefan W. Ryter,Augustine M.K. Choi +19 more
TL;DR: A critical role for Egr-1 is demonstrated in promoting autophagy and apoptosis in response to cigarette smoke exposure in vitro and in vivo, suggesting novel therapeutic targets for the treatment of cigarette smoke induced lung injury.