Protein Domain : Polycystic kidney disease type 1 protein IPR000434

Type	Family
Description	Polycystin-1 (PC1) plays a critical role in renal tubule diameter control. Mutations in the polycystin-1 gene cause cyst formation in human autosomal dominant polycystic kidney disease [ , ]. It may serve as a cell surface signaling receptor at cell-cell/cell-matrix junctions and as a mechano-sensor in renal primary cilia that activates signalling pathways involved in renal tubular differentiation [].Polycystin-1 contains an REJ (receptor for egg jelly) domain and a GPS (G protein-coupled receptor proteolytic site) domain in its N-terminal extracellular region (ectodomain). It can be cleaved into N-terminal fragment (NTF) and C-terminal fragment (CTF) at the GPS domain [ ]. The GPS cleavage may play an important role for the biological function of PC1 [].
Short Name	PC1