Article
CFTR processing, trafficking and interactions.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2020
Amaral Margarida D, Hutt Darren M, Tomati Valeria, Botelho Hugo M, Pedemonte Nicoletta
Abstract excerpt
Mutations associated with cystic fibrosis (CF) have complex effects on the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common CF mutation, F508del, disrupts the processing to and stability at the plasma membrane and function as a Cl- channel. CFTR is surrounded by a dynamic network of interacting components, referred to as the CFTR Functional Landscape, that impact its synthesis,...
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