[HTML][HTML] Characterization of cis-autoproteolysis of polycystin-1, the product of human polycystic kidney disease 1 gene

W Wei, K Hackmann, H Xu, G Germino… - Journal of Biological …, 2007 - ASBMB
Polycystin-1 (PC1), the PKD1 gene product, plays a critical role in renal tubule diameter
control and disruption of its function causes cyst formation in human autosomal dominant
polycystic kidney disease. Recent evidence shows that PC1 undergoes cleavage at the
juxtamembrane G protein-coupled receptor proteolytic site (GPS), a process likely to be
essential for its biological activity. Here we further characterized the proteolytic cleavage of
PC1 at the GPS domain. We determined the actual cleavage site to be between leucine and …