Rescue of Murine F508del CFTR Activity in Native Intestine by Low Temperature and Proteasome Inhibitors

Most patients with Cystic Fibrosis (CF) carry at least one allele with the F508del mutation, resulting in a CFTR chloride channel protein with a processing, gating and stability defect, but with substantial residual activity when correctly sorted to the apical membranes of epithelial cells. New ther...

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Detalles Bibliográficos
Autores principales: Wilke, Martina, Bot, Alice, Jorna, Huub, Scholte, Bob J., de Jonge, Hugo R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2012
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3528711/
https://www.ncbi.nlm.nih.gov/pubmed/23284872
http://dx.doi.org/10.1371/journal.pone.0052070

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3528711/
https://www.ncbi.nlm.nih.gov/pubmed/23284872
http://dx.doi.org/10.1371/journal.pone.0052070

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