Article
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth.
American journal of physiology. Renal physiology - 15 Oct 2012
Li Hongyu, Yang Wanding, Mendes Filipa, Amaral Margarida D, Sheppard David N
Abstract excerpt
In autosomal dominant polycystic kidney disease (ADPKD), cystic fibrosis transmembrane conductance regulator (CFTR), the protein product of the gene defective in cystic fibrosis (CF), plays a crucial role in fluid accumulation, which promotes cyst swelling. Several studies have identified individuals afflicted by both ADPKD and CF. Two studies suggested that CF mutations might attenuate the severity of ADPKD,...
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