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The most common cystic fibrosis-associated mutation destabilizes the dimeric state of the nucleotide-binding domains of CFTR

ABSTRACT: The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel that belongs to the ATP binding cassette (ABC) superfamily. The deletion of the phenylalanine 508 (ΔF508-CFTR) is the most common mutation among cystic fibrosis (CF) patients. The mutant channels present a...

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Autores principales: Jih, Kang-Yang, Li, Min, Hwang, Tzyh-Chang, Bompadre, Silvia G
Formato: Artigo
Lenguaje:Inglês
Publicado: Blackwell Science Inc 2011
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3112550/
https://ncbi.nlm.nih.gov/pubmed/21486785
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1113/jphysiol.2010.202861
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