Ouabain mimics low temperature rescue of F508del-CFTR in cystic fibrosis epithelial cells

Ouabain mimics low temperature rescue of F508del-CFTR in cystic fibrosis epithelial cells

ABSTRACT Most cases of cystic fibrosis (CF) are caused by the deletion of a single phenylalanine residue at position 508 of the cystic fibrosis transmembrane conductance regulator (CFTR). The mutant F508del-CFTR is retained in the endoplasmic reticulum and degraded, but can be induced by low tempera...

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Bibliographic Details
Main Authors: Donglei eZhang, Fabiana eCiciriello, Suzana eAnjos, Annamaria eCarissimo, Jie eLiao, Graeme eCarlile, Haouaria eBalghi, Renaud eRobert, Alberto eLuini, John eHanrahan, David eThomas
Format: Article
Language:English
Published: Frontiers Media S.A. 2012-10-01
Series:Frontiers in Pharmacology
Subjects:
Cystic Fibrosis
Ouabain
Microarray
trafficking
CFTR
hierarchical clustering
Online Access:http://journal.frontiersin.org/Journal/10.3389/fphar.2012.00176/full

Internet

http://journal.frontiersin.org/Journal/10.3389/fphar.2012.00176/full

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