Article
A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability.
Molecular pharmacology - 1 Jun 2009
Wellhauser Leigh, Kim Chiaw Patrick, Pasyk Stan, Li Canhui, Ramjeesingh Mohabir, Bear Christine E
Abstract excerpt
The deletion of Phe-508 (DeltaPhe508) constitutes the most prevalent of a number of mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) that cause cystic fibrosis (CF). This mutation leads to CFTR misfolding and retention in the endoplasmic reticulum, as well as impaired c...
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