瞬时受体电位阳离子通道亚家族M成员6：一种类两面神蛋白。

TRPM6: A Janus-like protein.

作者信息

Bödding M

机构信息

Experimentelle und Klinische Pharmakologie und Toxikologie, Universität des Saarlandes, 66421 Homburg, Germany.

出版信息

Handb Exp Pharmacol. 2007(179):299-311. doi: 10.1007/978-3-540-34891-7_18.

DOI:10.1007/978-3-540-34891-7_18

PMID:17217065

Abstract

TRPM6 and TRPM7 proteins share similar molecular structures and biophysical properties. Both proteins are Mg(2+)- and Ca(2+)-permeable cation channels with the typical topology of six transmembrane domains. In addition, TRPM6 and TRPM7 function as serine/threonine kinases with kinase domains at their C-terminal tails. At present, the role of the association of kinase and channel domains in TRPM6 and TRPM7 remains elusive. TRPM6 is mainly expressed in kidney and intestine, where it might be responsible for epithelial Mg2+ re/absorption. This hypothesis is strengthened by the identification of TRPM6 mutants in patients with a rare but severe hereditary disease called hypomagnesaemia with secondary hypocalcaemia. The aim of this review is to provide a brief but concise overview of the information currently available about TRPM6.

摘要

瞬时受体电位M6型（TRPM6）和瞬时受体电位M7型（TRPM7）蛋白具有相似的分子结构和生物物理特性。这两种蛋白都是镁离子（Mg²⁺）和钙离子（Ca²⁺）可通透的阳离子通道，具有典型的六个跨膜结构域拓扑结构。此外，TRPM6和TRPM7作为丝氨酸/苏氨酸激酶发挥作用，其C末端尾部带有激酶结构域。目前，TRPM6和TRPM7中激酶结构域与通道结构域结合的作用仍不清楚。TRPM6主要在肾脏和肠道中表达，可能在其中负责上皮细胞对镁离子的重吸收。一种罕见但严重的遗传性疾病——继发性低钙血症伴低镁血症患者中TRPM6突变体的发现，强化了这一假说。本综述的目的是简要而精炼地概述目前有关TRPM6的可用信息。