Processing and function of CFTR-ΔF508 are species-dependent

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The most common mutation, a deletion of the phenylalanine at position 508 (ΔF508), disrupts processing of the protein. Nearly all human CFTR-ΔF508 is retained in the endoplasmic reticulum and degraded,...

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Detalhes bibliográficos
Main Authors: Ostedgaard, Lynda S., Rogers, Christopher S., Dong, Qian, Randak, Christoph O., Vermeer, Daniel W., Rokhlina, Tatiana, Karp, Philip H., Welsh, Michael J.
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2007
Assuntos:
Biological Sciences
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1976592/
https://ncbi.nlm.nih.gov/pubmed/17873061
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0706974104
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