Diminished Self-Chaperoning Activity of the ΔF508 Mutant of CFTR Results in Protein Misfolding

The absence of a functional ATP Binding Cassette (ABC) protein called the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) from apical membranes of epithelial cells is responsible for cystic fibrosis (CF). Over 90% of CF patients carry at least one mutant allele with deletion of phenylalan...

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Autori principali: Serohijos, Adrian W. R., Hegedűs, Tamás, Riordan, John R., Dokholyan, Nikolay V.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Public Library of Science 2008
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Research Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2265529/
https://ncbi.nlm.nih.gov/pubmed/18463704
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pcbi.1000008
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