PKD2 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. Can also form a functional, homotetrameric ion channel. Functions as a cation channel involved in fluid-flow mechanosensation by the primary cilium in renal epithelium. Functions as outward-rectifying K(+) channel, but is also permeable to Ca(2+), and to a much lesser degree also to Na(+). May contribute to the release of Ca(2+) stores from the endoplasmic reticulum. Together with TRPV4, forms mechano- and thermosensitive channels in cilium. PKD1 and PKD2 may function through a common signaling pathway that is necessary to maintain the normal, differentiated state of renal tubule cells. Acts as a regulator of cilium length, together with PKD1. 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. Also involved in left-right axis specification via its role in sensing nodal flow; forms a complex with PKD1L1 in cilia to facilitate flow detection in left-right patterning. Detection of asymmetric nodal flow gives rise to a Ca(2+) signal that is required for normal, asymmetric expression of genes involved in the specification of body left-right laterality. Belongs to the polycystin family. Detected in fetal and adult kidney (PubMed:10770959). Detected at the thick ascending limb of the loop of Henle, at distal tubules, including the distal convoluted tubule and cortical collecting tubules, with weak staining of the collecting duct (PubMed:10770959). Detected on placenta syncytiotrophoblasts (at protein level) (PubMed:26269590). Strongly expressed in ovary, fetal and adult kidney, testis, and small intestine. Not detected in peripheral leukocytes. 5 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Channel, cation; Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 4q22.1
Cellular Component:  basal cortex; basal plasma membrane; cation channel complex; ciliary basal body; ciliary membrane; cilium; cytoplasm; cytoplasmic vesicle membrane; endoplasmic reticulum; endoplasmic reticulum membrane; Golgi apparatus; integral component of cytoplasmic side of endoplasmic reticulum membrane; integral component of lumenal side of endoplasmic reticulum membrane; integral component of plasma membrane; lamellipodium; mitotic spindle; motile cilium; non-motile cilium; plasma membrane; polycystin complex
Molecular Function:  actinin binding; ATPase binding; calcium ion binding; calcium-induced calcium release activity; cation channel activity; cytoskeletal protein binding; HLH domain binding; identical protein binding; ion channel binding; muscle alpha-actinin binding; outward rectifier potassium channel activity; phosphoprotein binding; potassium channel activity; protein binding; protein homodimerization activity; signaling receptor binding; voltage-gated calcium channel activity; voltage-gated cation channel activity; voltage-gated ion channel activity; voltage-gated potassium channel activity; voltage-gated sodium channel activity
Biological Process:  aorta development; branching involved in ureteric bud morphogenesis; calcium ion transmembrane transport; calcium ion transport; cell cycle arrest; cell-cell signaling by wnt; cellular response to calcium ion; cellular response to cAMP; cellular response to fluid shear stress; cellular response to hydrostatic pressure; cellular response to osmotic stress; cellular response to reactive oxygen species; centrosome duplication; cilium organization; cytoplasmic sequestering of transcription factor; detection of mechanical stimulus; detection of nodal flow; determination of left/right symmetry; determination of liver left/right asymmetry; embryonic placenta development; heart development; heart looping; inorganic cation transmembrane transport; JAK-STAT cascade; liver development; mesonephric duct development; mesonephric tubule development; metanephric ascending thin limb development; metanephric cortex development; metanephric cortical collecting duct development; metanephric distal tubule development; metanephric mesenchyme development; metanephric part of ureteric bud development; metanephric S-shaped body morphogenesis; metanephric smooth muscle tissue development; negative regulation of cell proliferation; negative regulation of G1/S transition of mitotic cell cycle; negative regulation of ryanodine-sensitive calcium-release channel activity; neural tube development; placenta blood vessel development; positive regulation of cell cycle arrest; positive regulation of cyclin-dependent protein serine/threonine kinase activity; positive regulation of inositol 1,4,5-trisphosphate-sensitive calcium-release channel activity; positive regulation of nitric oxide biosynthetic process; positive regulation of transcription by RNA polymerase II; potassium ion transmembrane transport; protein heterotetramerization; protein homotetramerization; protein tetramerization; regulation of calcium ion import; regulation of cell proliferation; release of sequestered calcium ion into cytosol; renal artery morphogenesis; renal tubule morphogenesis; sodium ion transmembrane transport; spinal cord development; Wnt signaling pathway
Disease: Polycystic Kidney Disease 2
Reference #:  Q13563 (UniProtKB)
Alt. Names/Synonyms: APKD2; Autosomal dominant polycystic kidney disease type II protein; MGC138466; MGC138468; Pc-2; PC2; PKD2; PKD4; polycystic kidney disease 2 (autosomal dominant); Polycystic kidney disease 2 protein; polycystin 2, transient receptor potential cation channel; Polycystin-2; Polycystwin; R48321; Transient receptor potential cation channel subfamily P member 2; transient receptor potential cation channel, subfamily P, member 2; TRPP2
Gene Symbols: PKD2
Molecular weight: 109,691 Da
Basal Isoelectric point: 5.49  Predict pI for various phosphorylation states
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Protein Structure Not Found.

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