Polycystin-1/PKD1 Products

Description

Polycystin-1 (also PKD1) is a 500-550 kDa member of the polycystin family of proteins. It is expressed in renal tubule primary cilia, and the membrane region that forms adherens junctions. Polycystin-1 binds to polycystin-2, promoting its insertion into the cell membrane, and regulating its calcium channel activity. In conjunction with polycystin-2, it detects fluid flow and converts this information into calcium signals. It also exists in the ER, where it negatively modulates polycystin-2 mediated calcium release. Mature human polycystin-1 is a 4280 amino acid (aa), 11 transmembrane glycoprotein. Its N-terminal extracellular region (aa 24-3074) is highly modular, and contains a C-type lecin domain, multiple Leu-rich repeats and PKD domains, one GPS region and a PLAT, REJ, WSC and LDLR domain. The cytoplasmic C-terminus (aa 4107-4303) contains a polycystin-2 coiled-coil interaction domain (aa 4220-4251). A percentage of polycystin-1 undergoes autoproteolysis after Leu3048, generating a presumed N-terminal fragment of ~ 350-400 kDa, and a membrane-bound C-terminal fragment of 150 kDa. Over aa 4141-4303, human polycystin-1 shares 71% aa identity with mouse polycystin-1.

Bioinformatics

Entrez	5310 Human 18763 Mouse 24650 Rat
Product By Gene ID	5310
Alternate Names	Autosomal dominant polycystic kidney disease 1 protein PBP Pc-1 PKD1 polycystic kidney disease 1 (autosomal dominant) polycystic kidney disease-associated protein polycystin 1 Polycystin1 Polycystin-1 transient receptor potential cation channel, subfamily P, member 1 TRPP1

Research Areas for Polycystin-1/PKD1

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Phospho-Specific