Article
Cleavage of polycystin-1 requires the receptor for egg jelly domain and is disrupted by human autosomal-dominant polycystic kidney disease 1-associated mutations.
Proceedings of the National Academy of Sciences of the United States of America - 24 Dec 2002
Qian Feng, Boletta Alessandra, Bhunia Anil K, Xu Hangxue, Liu Lijuan, Ahrabi Ali K, Watnick Terry J, Zhou Fang, Germino Gregory G
Abstract excerpt
Polycystin-1 plays an essential role in renal tubular morphogenesis, and disruption of its function causes cystogenesis in human autosomal-dominant polycystic kidney disease (ADPKD). We demonstrated that polycystin-1 undergoes cleavage at G protein coupled receptor proteolytic site in a process that requires the receptor for egg jelly domain. Most of the N-terminal fragment remains tethered at the cell surface,...
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