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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Veröffentlicht in:	PloS one 2012-12, Vol.7 (12), p.e52070-e52070
Hauptverfasser:	Wilke, Martina, Bot, Alice, Jorna, Huub, Scholte, Bob J, de Jonge, Hugo R
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Autophagy Biological Transport - drug effects Biology Bortezomib Brefeldin A C band Cancer Cell culture Cell cycle Channel gating Chloride Chlorides Chlorides - metabolism Cold Temperature Consortia Crypts Cystic fibrosis Cystic Fibrosis - genetics Cystic Fibrosis - metabolism Cystic fibrosis transmembrane conductance regulator Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Defects Epithelial cells Epithelium Fibrosis Forskolin Gastroenterology Golgi apparatus Ileum Incubation Inhibitors Intestinal Mucosa - drug effects Intestinal Mucosa - metabolism Intestine Intestines - drug effects Intestines - metabolism Laboratory animals Low temperature Medicine Membranes Mice Mice, Knockout Multiple myeloma Muscles Mutation Proteasome inhibitors Proteasome Inhibitors - pharmacology Proteins Short circuits Short-circuit current
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