Cystic fibrosis: the 'bicarbonate before chloride' hypothesis.
The specific effects of some mutations that cause cystic fibrosis suggest that reduced HCO(3)(-) transport is the key to understanding cystic fibrosis pathology. But there is a puzzling discrepancy between measures of CFTR-mediated chloride conductance in expression systems and the sweat chloride values of patients.
Wine
J J
JJ
Cystic Fibrosis Research Laboratory, Stanford University, Stanford, California 94305-2130, USA.
D001639:Bicarbonates / Q000378:metabolism*; D001692:Biological Transport / Q000235:genetics / Q000502:physiology; D002712:Chlorides / Q000378:metabolism*; D003550:Cystic Fibrosis / Q000235:genetics* / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D013542:Sweat / Q000737:chemistry*
D001639:Bicarbonates; C505032:CFTR protein, human; D002712:Chlorides; D019005:Cystic Fibrosis Transmembrane Conductance Regulator