Essential role of cleavage of Polycystin-1 at G protein-coupled receptor proteolytic site for kidney tubular structure

S Yu, K Hackmann, J Gao, X He… - Proceedings of the …, 2007 - National Acad Sciences
Polycystin-1 (PC1) has an essential function in renal tubular morphogenesis and disruption
of its function causes cystogenesis in human autosomal dominant polycystic kidney disease.
We have previously shown that recombinant human PC1 is cis-autoproteolytically cleaved at
the G protein-coupled receptor proteolytic site domain. To investigate the role of cleavage in
vivo, we generated by gene targeting a Pkd1 knockin mouse (Pkd1 V/V) that expresses
noncleavable PC1. The Pkd1 V/V mice show a hypomorphic phenotype, characterized by a …

[引用][C] Essential role of cleavage of Polycystin-1 at G protein-coupled receptor proteolytic site for kidney tubular structure (Proceedings of the National Academy of …

S Yu, K Hackmann, J Gao, X He… - Proceedings of the …, 2008 - pure.johnshopkins.edu
Essential role of cleavage of Polycystin-1 at G protein-coupled receptor proteolytic site for
kidney tubular structure (Proceedings of the National Academy of Sciences of the United
States of America (2007) 104, (18688-18693) DOI: 10.1073/pnas.0708217104) — Johns
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