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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Detalles Bibliográficos
Autores principales: Hoelen, Hanneke, Kleizen, Bertrand, Schmidt, Andre, Richardson, John, Charitou, Paraskevi, Thomas, Philip J., Braakman, Ineke
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2010
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2994901/
https://www.ncbi.nlm.nih.gov/pubmed/21152102
http://dx.doi.org/10.1371/journal.pone.0015458

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