Pharmacological correctors of mutant CFTR mistrafficking

The lack of phenylalanine 508 (∆F508 mutation) in the CFTR Cl- channel represents the most frequent cause of cystic fibrosis (CF), a genetic disease affecting multiple organs such lung, pancreas, and liver. ∆F508 causes instability and misfolding of CFTR protein leading to early degradation in the...

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Bibliographic Details
Main Authors: Nicoletta ePedemonte, Luis eGalietta
Format: Article
Language:English
Published: Frontiers Media S.A. 2012-10-01
Series:Frontiers in Pharmacology
Subjects:
Online Access:http://journal.frontiersin.org/Journal/10.3389/fphar.2012.00175/full