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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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Detalles Bibliográficos
Autores principales: 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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4102440/
https://www.ncbi.nlm.nih.gov/pubmed/25033378
http://dx.doi.org/10.1371/journal.pgen.1004376