HCO
3- and water in the pancreatic juice is secreted by ductal epithelium. Mechanisms for apical HCO
3- transport differ dependent on luminal anion composition along the ductal tree, but they are entirely dependent on cystic fibrosis transmembrane conductance regulator (CFTR) Cl
- channel. This HCO
3--rich fluid secretion effectively washes out protein-rich acinar secretion, which is impaired in cystic fibrosis (CF) and a certain cases of chronic pancreatitis. Idiopathic chronic pancreatitis (ICP) is known as atypical/nonclassic CF and develops in compound heterozygote of severe/mild mutations of CFTR. Recent further analysis of CFTR mutations/polymorphisms and sweat Cl
- revealed that CF carrier status (in Caucasian) and combination of polymorphisms (in Japanese) are attributable risk for ICP, which is related to mild dysfunction of CFTR.
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