Recombinant Human Polycystin-2 (PKD2), Truncated

Uniprot Id

Q13563

Target Species

Human

Target Name

PKD2

Target Full Name

Polycystin-2

Target Function

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.

Target Involvement

Polycystic kidney disease 2 (PKD2)

Target Subcellular Location

Cell projection, cilium membrane; Multi-pass membrane protein. Endoplasmic reticulum membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein. Basolateral cell membrane. Cytoplasmic vesicle membrane. Golgi apparatus.

Target Protein Families

Polycystin family

Target Tissue Specificity

Detected in fetal and adult kidney. 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. Detected on placenta syncy

Target Synonyms

APKD2; Autosomal dominant polycystic kidney disease type II; Autosomal dominant polycystic kidney disease type II protein; MGC138466; MGC138468; PC 2; PC2; PKD 2; PKD2; PKD2_HUMAN; PKD4; Polycystic kidney disease 2 (autosomal dominant); Polycystic kidney disease 2; Polycystic kidney disease 2 protein; Polycystin 2; Polycystin 2 transient receptor potential cation channel; Polycystin-2; Polycystin2; Polycystwin; R48321; Transient receptor potential cation channel subfamily P member 2; TRPP2

Target Background

This gene encodes a member of the polycystin protein family. The encoded protein is a multi-pass membrane protein that functions as a calcium permeable cation channel, and is involved in calcium transport and calcium signaling in renal epithelial cells. This protein interacts with polycystin 1, and they may be partners in a common signaling cascade involved in tubular morphogenesis. Mutations in this gene are associated with autosomal dominant polycystic kidney disease type 2.