Mechanisms of CFTR functional variants that impair regulated bicarbonate permeation and increase risk for pancreatitis but not for cystic fibrosis.

Mechanisms of CFTR functional variants that impair regulated bicarbonate permeation and increase risk for pancreatitis but not for cystic fibrosis.

CFTR is a dynamically regulated anion channel. Intracellular WNK1-SPAK activation causes CFTR to change permeability and conductance characteristics from a chloride-preferring to bicarbonate-preferring channel through unknown mechanisms. Two severe CFTR mutations (CFTRsev) cause complete loss of CFT...

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Bibliographic Details
Main Authors: Jessica LaRusch, Jinsei Jung, Ignacio J General, Michele D Lewis, Hyun Woo Park, Randall E Brand, Andres Gelrud, Michelle A Anderson, Peter A Banks, Darwin Conwell, Christopher Lawrence, Joseph Romagnuolo, John Baillie, Samer Alkaade, Gregory Cote, Timothy B Gardner, Stephen T Amann, Adam Slivka, Bimaljit Sandhu, Amy Aloe, Michelle L Kienholz, Dhiraj Yadav, M Michael Barmada, Ivet Bahar, Min Goo Lee, David C Whitcomb, North American Pancreatitis Study Group
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-07-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC4102440?pdf=render

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