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Increased folding and channel activity of a rare cystic fibrosis mutant with CFTR modulators

Cystic fibrosis (CF) is a lethal recessive genetic disease caused by mutations in the CFTR gene. The gene product is a PKA-regulated anion channel that is important for fluid and electrolyte transport in the epithelia of lung, gut, and ducts of the pancreas and sweat glands. The most common CFTR mut...

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Detalhes bibliográficos
Main Authors: Caldwell, Ray A., Grove, Diane E., Houck, Scott A., Cyr, Douglas M.
Formato: Artigo
Idioma:Inglês
Publicado em: American Physiological Society 2011
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3174745/
https://ncbi.nlm.nih.gov/pubmed/21642448
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajplung.00044.2011
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