Protein Domain : IPR000434

Type:  Family Name:  Polycystic kidney disease type 1 protein
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
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0 Child Features

4 Contains

DB identifier Type Name
IPR001024 Domain PLAT/LH2 domain
IPR013122 Domain Polycystin cation channel, PKD1/PKD2
IPR000483 Domain Cysteine-rich flanking region, C-terminal
IPR000372 Domain Leucine-rich repeat N-terminal domain

1 Cross References

Identifier
PR00500

0 Found In

2 GO Annotations

GO Term Gene Name
GO:0001822 IPR000434
GO:0016021 IPR000434

2 Ontology Annotations

GO Term Gene Name
GO:0001822 IPR000434
GO:0016021 IPR000434

0 Parent Features

1 Proteins

DB identifier UniProt Accession Secondary Identifier Organism Name Length
Solyc12g062960.1.1 PAC:36148031 Solanum lycopersicum 618  

4 Publications

First Author Title Year Journal Volume Pages PubMed ID
            12482949
            17525154
            8554072
            22508176