Physiology of epithelial chloride and fluid secretion.
Epithelial salt and water secretion serves a variety of functions in different organ systems, such as the airways, intestines, pancreas, and salivary glands. In cystic fibrosis (CF), the volume and/or composition of secreted luminal fluids are compromised owing to mutations in the gene encoding CFTR, the apical membrane anion channel that is responsible for salt secretion in response to cAMP/PKA stimulation. This article examines CFTR and related cellular transport processes that underlie epithelial anion and fluid secretion, their regulation, and how these processes are altered in CF disease to account for organ-specific secretory phenotypes.
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. frizzell@pitt.edu
Raymond A
RA
Frizzell
John W
JW
Hanrahan
D002712:Chlorides / Q000378:metabolism*; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism*; D004847:Epithelial Cells / Q000378:metabolism*; D018495:Fluid Shifts / Q000502:physiology; D006801:Humans; D014882:Water-Electrolyte Balance / Q000502:physiology*
D002712:Chlorides; D019005:Cystic Fibrosis Transmembrane Conductance Regulator