Zobrazeno 1 - 10
of 153
pro vyhledávání: '"Michael J. Welsh"'
Autor:
Tayyab Rehman, Michael J. Welsh
Publikováno v:
Cells, Vol 12, Iss 8, p 1104 (2023)
The airway surface liquid (ASL) is a thin sheet of fluid that covers the luminal aspect of the airway epithelium. The ASL is a site of several first-line host defenses, and its composition is a key factor that determines respiratory fitness. Specific
Externí odkaz:
https://doaj.org/article/2c2a4a640ff848d3977a6506351fa837
Autor:
Samantha L. Durfey, Sudhakar Pipavath, Anna Li, Anh T. Vo, Anina Ratjen, Suzanne Carter, Sarah J. Morgan, Matthew C. Radey, Brenda Grogan, Stephen J. Salipante, Michael J. Welsh, David A. Stoltz, Christopher H. Goss, Edward F. McKone, Pradeep K. Singh
Publikováno v:
mBio, Vol 12, Iss 6 (2021)
ABSTRACT Drugs called CFTR modulators improve the physiologic defect underlying cystic fibrosis (CF) and alleviate many disease manifestations. However, studies to date indicate that chronic lung infections that are responsible for most disease-relat
Externí odkaz:
https://doaj.org/article/6014c178cf2145dba42af057cdbac508
Autor:
Tayyab Rehman, Philip H. Karp, Andrew L. Thurman, Steven E. Mather, Akansha Jain, Ashley L. Cooney, Patrick L. Sinn, Alejandro A. Pezzulo, Michael E. Duffey, Michael J. Welsh
Publikováno v:
American Journal of Respiratory Cell and Molecular Biology. 67:491-502
In cystic fibrosis (CF), reduced HCOsub3/subsup-/supsecretion acidifies the airway surface liquid (ASL), and the acidic pH disrupts host defenses. Thus, understanding the control of ASL pH (pHsubASL/sub) in CF may help identify novel targets and faci
Autor:
Andrew L. Thurman, Xiaopeng Li, Raul Villacreses, Wenjie Yu, Huiyu Gong, Steven E. Mather, Guillermo S. Romano-Ibarra, David K. Meyerholz, David A. Stoltz, Michael J. Welsh, Ian M. Thornell, Joseph Zabner, Alejandro A. Pezzulo
Publikováno v:
American journal of respiratory cell and molecular biology. 66(6)
Lack of CFTR (cystic fibrosis transmembrane conductance regulator) affects the transcriptome, composition, and function of large and small airway epithelia in people with advanced cystic fibrosis (CF); however, whether lack of CFTR causes cell-intrin
Autor:
Michael J. Welsh, Janice L. Launspach, Martin D. Burke, Rajeev S. Chorghade, Philip H. Karp, Bo Ram Kim
Publikováno v:
J Cyst Fibros
Background Approximately 10% of people with cystic fibrosis (CF) have mutations that result in little to no CFTR production and thus cannot benefit from CFTR modulators. We previously found that Amphotericin B (AmB), a small molecule that forms anion
Autor:
Maria I. Pino-Argumedo, Anthony J. Fischer, Brieanna M. Hilkin, Nicholas D. Gansemer, Patrick D. Allen, Eric A. Hoffman, David A. Stoltz, Michael J. Welsh, Mahmoud H. Abou Alaiwa
Publikováno v:
Proceedings of the National Academy of Sciences of the United States of America. 119(13)
SignificanceIn many lung diseases, increased amounts of and/or abnormal mucus impair mucociliary clearance, a key defense against inhaled and aspirated material. Submucosal glands lining cartilaginous airways secrete mucus strands that are pulled by
Publikováno v:
The Journal of Physiology. 598:4307-4320
Key points Cl- and HCO3 - had similar paracellular permeabilities in human airway epithelia. PCl /PNa of airway epithelia was unaltered by pH 7.4 vs. pH 6.0 solutions. Under basal conditions, calculated paracellular HCO3 - flux was secretory. Cytokin
Autor:
Alicia K. Gerke, Aaron C. Miller, Joseph E. Cavanaugh, Douglas B. Hornick, Alejandro P. Comellas, David A. Stoltz, Michael J. Welsh, Joseph Zabner, Philip M. Polgreen
Publikováno v:
Proceedings of the National Academy of Sciences. 117:1621-1627
Autosomal recessive diseases, such as cystic fibrosis (CF), require inheritance of 2 mutated genes. However, some studies indicate that CF carriers are at increased risk for some conditions associated with CF. These investigations focused on single c
Autor:
Ping Tan, Alejandro A. Pezzulo, Tayyab Rehman, Ian M. Thornell, Michael J. Welsh, S.L. Durfey, Andrew L Thurman, Brian J Goodell, Edward F. McKone, David A. Stoltz, Michael E. Duffey, Philip H. Karp, Pradeep K. Singh
Publikováno v:
J Clin Invest
Without cystic fibrosis transmembrane conductance regulator–mediated (CFTR-mediated) HCO(3)(–) secretion, airway epithelia of newborns with cystic fibrosis (CF) produce an abnormally acidic airway surface liquid (ASL), and the decreased pH impair
Autor:
Bo Ram Kim, Alexander G. Cioffi, Michael J. Welsh, Katrina A. Muraglia, Martin D. Burke, Lingyang Zhu, Viral Shah, Page N. Daniels, Philip H. Karp, Rajeev S. Chorghade, Xiao Xiao Tang, Anthony S. Grillo
Publikováno v:
Nature
Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) compromise epithelial HCO3− and Cl− secretion, reduce airway surface liquid pH, and impair respiratory host defences in people with cystic fibrosis1–3.