Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein

Most cystic fibrosis is caused by the deletion of a single amino acid (F508) from CFTR and the resulting misfolding and destabilization of the protein. Compounds identified by high-throughput screening to improve ΔF508 CFTR maturation have already entered clinical trials, and it is important to unde...

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Bibliografiske detaljer
Main Authors: He, Lihua, Kota, Pradeep, Aleksandrov, Andrei A., Cui, Liying, Jensen, Tim, Dokholyan, Nikolay V., Riordan, John R.
Format: Artigo
Sprog:Inglês
Udgivet: Federation of American Societies for Experimental Biology 2013
Fag:
Research Communications
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3545534/
https://ncbi.nlm.nih.gov/pubmed/23104983
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.12-216119
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