VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1

Cystic fibrosis (CF) is a fatal genetic disorder associated with defective hydration of lung airways due to the loss of chloride transport through the CF transmembrane conductance regulator protein (CFTR). CFTR contains two membrane-spanning domains (MSDs), two nucleotide-binding domains (NBDs), and...

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Main Authors: Ren, Hong Yu, Grove, Diane E., De La Rosa, Oxana, Houck, Scott A., Sopha, Pattarawut, Van Goor, Fredrick, Hoffman, Beth J., Cyr, Douglas M.
Format: Artigo
Jezik:Inglês
Izdano: The American Society for Cell Biology 2013
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Articles
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC3784376/
https://ncbi.nlm.nih.gov/pubmed/23924900
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1091/mbc.E13-05-0240
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