Restoration of NBD1 thermal stability is necessary and sufficient to correct ΔF508 CFTR folding and assembly

CFTR (ABCC7), unique among ABC exporters as an ion channel, regulates ion and fluid transport in epithelial tissues. Loss of function due to mutations in the cftr gene causes cystic fibrosis (CF). The most common CF-causing mutation, the deletion of F508 (ΔF508) from the first nucleotide binding dom...

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Bibliographic Details
Published in:J Mol Biol
Main Authors: He, Lihua, Aleksandrov, Andrei A, An, Jianli, Cui, Liying, Yang, Zhengrong, Brouillette, Christie G., Riordan, John R
Format: Artigo
Language:Inglês
Published: 2014
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Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC4757845/
https://ncbi.nlm.nih.gov/pubmed/25083918
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jmb.2014.07.026
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