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Functions as a calcium permeable cation channel involved in fluid-flow mechanosensation by the primary cilium in renal epithelium. PKD1 and PKD2 may function through a common signaling pathway that is necessary for normal tubulogenesis. 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 downstream of nodal flow: forms a complex with PKD2 in cilia to facilitate flow detection in left/right patterning.
Gene Name: | polycystic kidney disease 2 (autosomal dominant) |
Family/Subfamily: | Ion Channel , Calcium channel - TRP |
Synonyms: | PKD2, APKD2, Pc-2, Polycystic kidney disease 2, Polycystwin, PC2, PKD4, Polycystin-2, R48321, TRPP2 |
Target Sequences: | NM_000297 NP_000288.1 Q13563 |
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