Zobrazeno 1 - 10
of 81
pro vyhledávání: '"Michael J. Welsh"'
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:
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.
Autor:
Erica N. LeClair, David A. Stoltz, Nicholas D. Gansemer, Katherine N. Gibson-Corley, Paul B. McCray, Michael J. Welsh, Mariah R. Leidinger, Sarah E. Ernst, Carrie K. Barker, Matthew D. Strub, Ryan J. Adam, Daniel P. Cook, Philip H. Karp, David K. Meyerholz
Publikováno v:
Laboratory investigation; a journal of technical methods and pathology
Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function causes cystic fibrosis (CF), predisposing the lungs to chronic infection and inflammation. In young infants with CF, structural airway defects are increasingly recognized bef
Autor:
Michael J. Welsh, Carrie K. Barker, David K. Meyerholz, David A. Stoltz, Alejandro A. Pezzulo, Joseph Zabner, Jennifer A. Bartlett, Christine L. Wohlford-Lenane, Shyam Ramachandran, Paul B. McCray
Publikováno v:
American Journal of Respiratory and Critical Care Medicine. 194:845-854
Studies suggest that inappropriate responses to proinflammatory stimuli might contribute to inflammation in cystic fibrosis (CF) lungs. However, technical challenges have made it difficult to distinguish whether altered responses in CF airways are an
Autor:
David A. Stoltz, Michael V. Rector, Kathryn Chaloner, Philip H. Karp, Leah R. Reznikov, Jeng-Haur Chen, Alejandro A. Pezzulo, Sarah E. Ernst, Janice L. Launspach, Thomas O. Moninger, Michael J. Welsh, Joseph Zabner
Publikováno v:
Cell. 143(6):911-923
Defective transepithelial electrolyte transport is thought to initiate cystic fibrosis (CF) lung disease. Yet, how loss of CFTR affects electrolyte transport remains uncertain. CFTR -/- pigs spontaneously develop lung disease resembling human CF. At
Publikováno v:
Proceedings of the National Academy of Sciences. 106:3591-3596
Electrolyte transport through and between airway epithelial cells controls the quantity and composition of the overlying liquid. Many studies have shown acute regulation of transcellular ion transport in airway epithelia. However, whether ion transpo
Autor:
Qian Dong, Viral Shah, Michael J. Welsh, Amanda R. Ver Heul, Sarah E. Ernst, Lynda S. Ostedgaard, Christoph O. Randak
Publikováno v:
The Journal of Biological Chemistry
Background: Cystic fibrosis transmembrane conductance regulator (CFTR) channels have ATPase and adenylate kinase activity. Results: Mutating Gln-1291 disrupts adenylate kinase- but not ATPase-dependent gating, and it reduces channel activity in airwa
Autor:
Todd E. Scheetz, Tamara A. Kucaba, Justin Coco, Michael J. Welsh, Joseph Zabner, Mari E. Eyestone, Thomas L. Casavant, Paul B. McCray, Maria de Fatima Bonaldo, M. Bento Soares
Publikováno v:
Physiological Genomics. 17:69-77
The airway epithelium represents an important barrier between the host and the environment. It is a first site of contact with pathogens, particulates, and other stimuli, and has evolved the means to dynamically respond to these challenges. In an eff
Publikováno v:
Human Gene Therapy. 10:603-613
Gene transfer could provide a novel treatment for cystic fibrosis. However, current vectors, including recombinant adenoviruses, are relatively inefficient at gene transfer to airway epithelia. We have found that delivering adenovirus in a calcium ph
Publikováno v:
Journal of Biological Chemistry. 273:22693-22700
Three subunits, alpha, beta, and gamma, contribute to the formation of the epithelial Na+ channel. To investigate the oligomeric assembly of the channel complex, we used sucrose gradient sedimentation analysis to determine the sedimentation propertie