PKD2 Q13563

多囊蛋白-2 (PC2) (常染色体显性多囊肾病II型蛋白) (多囊肾病2型蛋白) (Polycystwin) (R48321) (瞬时受体电位阳离子通道亚科P成员2)

功能描述

形成非选择性阳离子通道 (PubMed:11854751, PubMed:11991947, PubMed:15692563, PubMed:26269590, PubMed:27071085, PubMed:31441214, PubMed:39009345)。可作为同源四聚体离子通道发挥作用，或与PKD1形成异聚体 (PubMed:31441214, PubMed:33164752)。根据其亚细胞定位及其结合伴侣的调节，表现出不同的功能 (PubMed:11854751, PubMed:11991947, PubMed:27214281, PubMed:29899465)。在初级纤毛中作为阳离子通道发挥作用，对单价阳离子的偏好优于二价阳离子，允许K(+)、Na(+)和Ca(2+)内流，对Ca(2+)的选择性较低 (PubMed:27071085)。参与肾上皮初级纤毛的液流机械感觉 (By similarity)。在内质网中，可能作为K(+)通道发挥作用以促进Ca(2+)释放 (By similarity)。异源四聚体PKD1/PKD2通道比同源PKD2通道具有更高的Ca(2+)通透性，并作为主要的Ca(2+)可通透通道发挥作用 (PubMed:31441214)。与其他许多通道（如TRPV4、TRPC1、IP3R、RYR2）相互作用并作为其调节因子，最终进一步影响细胞内信号传导，以调节细胞内Ca(2+)信号传导 (PubMed:11854751, PubMed:11991947, PubMed:27214281, PubMed:29899465)。与TRPV4一起，在纤毛中形成机械敏感和热敏感通道 (PubMed:18695040)。在心肌细胞中，PKD2通过直接结合调节受刺激RYR2受体的Ca(2+)释放 (By similarity)。还通过其在感知结节流中的作用参与左右轴特化；与PKD1L1在纤毛中形成复合物以促进左右模式形成中的流动检测 (By similarity)。与PKD1一起作为纤毛长度的调节因子发挥作用 (By similarity)。通过血管收缩介导全身血压并有助于脑动脉的肌源性反应 (By similarity)。 {ECO:0000250|UniProtKB:O35245, ECO:0000269|PubMed:11854751, ECO:0000269|PubMed:11991947, ECO:0000269|PubMed:15692563, ECO:0000269|PubMed:18695040, ECO:0000269|PubMed:26269590, ECO:0000269|PubMed:27071085, ECO:0000269|PubMed:27214281, ECO:0000269|PubMed:29899465, ECO:0000269|PubMed:31441214, ECO:0000269|PubMed:33164752, ECO:0000269|PubMed:39009345}。

组织特异性

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.

亚细胞定位

Cell projection, cilium membrane