The Primary Folding Defect and Rescue of ΔF508 CFTR Emerge during Translation of the Mutant Domain

In the vast majority of cystic fibrosis (CF) patients, deletion of residue F508 from CFTR is the cause of disease. F508 resides in the first nucleotide binding domain (NBD1) and its absence leads to CFTR misfolding and degradation. We show here that the primary folding defect arises during synthesis...

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Detalhes bibliográficos
Main Authors: Hoelen, Hanneke, Kleizen, Bertrand, Schmidt, Andre, Richardson, John, Charitou, Paraskevi, Thomas, Philip J., Braakman, Ineke
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2010
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2994901/
https://ncbi.nlm.nih.gov/pubmed/21152102
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0015458
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