Article
Rescue of murine F508del CFTR activity in native intestine by low temperature and proteasome inhibitors.
PloS one - 1 Jan 2012
Wilke Martina, Bot Alice, Jorna Huub, Scholte Bob J, de Jonge Hugo R
Abstract excerpt
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 t...
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