PKD1 Component of a heteromeric calcium-permeable ion channel formed by PKD1 and PKD2 that is activated by interaction between PKD1 and a Wnt family member, such as WNT3A and WNT9B. Both PKD1 and PKD2 are required for channel activity. Involved in renal tubulogenesis. Involved in fluid-flow mechanosensation by the primary cilium in renal epithelium. Acts as a regulator of cilium length, together with PKD2. The dynamic control of cilium length is essential in the regulation of mechanotransductive signaling. The cilium length response creates a negative feedback loop whereby fluid shear-mediated deflection of the primary cilium, which decreases intracellular cAMP, leads to cilium shortening and thus decreases flow-induced signaling. May be an ion-channel regulator. Involved in adhesive protein-protein and protein-carbohydrate interactions. Belongs to the polycystin family. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Cell adhesion; Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 16p13.3
Cellular Component:  basolateral plasma membrane; calcium channel complex; cation channel complex; cell surface; ciliary membrane; cilium; cytoplasm; endoplasmic reticulum; extracellular exosome; Golgi apparatus; Golgi membrane; Golgi-associated vesicle membrane; integral component of membrane; integral component of plasma membrane; lateral plasma membrane; motile cilium; nucleus; plasma membrane; polycystin complex
Molecular Function:  calcium channel activity; carbohydrate binding; ion channel binding; protein binding; protein domain specific binding; protein kinase binding; Wnt-activated receptor activity
Biological Process:  anatomical structure morphogenesis; branching morphogenesis of an epithelial tube; calcium ion transmembrane transport; calcium-independent cell-matrix adhesion; cartilage condensation; cartilage development; cell cycle arrest; cell-cell signaling by wnt; cell-matrix adhesion; cytoplasmic sequestering of transcription factor; detection of mechanical stimulus; digestive tract development; embryonic placenta development; establishment of cell polarity; genitalia development; heart development; homophilic cell adhesion via plasma membrane adhesion molecules; in utero embryonic development; JAK-STAT cascade; kidney development; liver development; lung epithelium development; lymph vessel morphogenesis; mesonephric duct development; mesonephric tubule development; metanephric ascending thin limb development; metanephric collecting duct development; metanephric distal tubule morphogenesis; metanephric proximal tubule development; neural tube development; peptidyl-serine phosphorylation; placenta blood vessel development; positive regulation of cyclin-dependent protein serine/threonine kinase activity; positive regulation of cytosolic calcium ion concentration; positive regulation of protein binding; positive regulation of transcription by RNA polymerase II; protein export from nucleus; protein heterotetramerization; regulation of cell adhesion; regulation of G1/S transition of mitotic cell cycle; regulation of mitotic spindle organization; regulation of proteasomal protein catabolic process; response to fluid shear stress; skin development; spinal cord development; Wnt signaling pathway
Disease: Polycystic Kidney Disease 1
Reference #:  P98161 (UniProtKB)
Alt. Names/Synonyms: Autosomal dominant polycystic kidney disease 1 protein; PBP; Pc-1; PC1; PKD1; polycystic kidney disease 1 (autosomal dominant); polycystic kidney disease-associated protein; polycystin 1; polycystin 1, transient receptor potential channel interacting; Polycystin-1; transient receptor potential cation channel, subfamily P, member 1; TRPP1
Gene Symbols: PKD1
Molecular weight: 462,529 Da
Basal Isoelectric point: 6.28  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene  |  InnateDB  |  Ensembl Protein