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 (CFTR(sev)) cause complete loss of C...

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Main Authors: LaRusch, Jessica, Jung, Jinsei, General, Ignacio J., Lewis, Michele D., Park, Hyun Woo, Brand, Randall E., Gelrud, Andres, Anderson, Michelle A., Banks, Peter A., Conwell, Darwin, Lawrence, Christopher, Romagnuolo, Joseph, Baillie, John, Alkaade, Samer, Cote, Gregory, Gardner, Timothy B., Amann, Stephen T., Slivka, Adam, Sandhu, Bimaljit, Aloe, Amy, Kienholz, Michelle L., Yadav, Dhiraj, Barmada, M. Michael, Bahar, Ivet, Lee, Min Goo, Whitcomb, David C.
Format: Artigo
Jezik:Inglês
Izdano: Public Library of Science 2014
Teme:
Research Article
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC4102440/
https://ncbi.nlm.nih.gov/pubmed/25033378
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pgen.1004376
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