KCNQ钾通道研究进展

KCNQ钾通道是钾通道超家族的一个重要分支,按其结构特性主要分为5大类:KCNQ1、KCNQ2、KCNQ3、KCNQ4、KCNQ5。它们不仅参与机体许多重要生理功能的调节,而且在一些疾病中还具有重要作用。KCNQ1是心肌I_(LS)电流的α亚单位,KCNQ1突变可导致长QT综合征(LQT综合征),KCNQ2/3、KCNQ3/5异源多聚体是M电流的分子基础,KCNQ2/3突变可诱发癫痫,KCNQ4编码的通道蛋白是外耳毛细胞I_(K.N)电流的分子基础,KCNQ4突变可导致先天性耳聋。

Research progress in KCNQ potassium channels

KCNQ channels are an important sub-family in potassium channels. They are divided into five subtypes, including KCNQ1, KCNQ2, KCNQ3, KCNQ4, KCNQ5. KCNQ channels have a wide range of physiological and pathophysiologi-cal correlates. KCNQ1 (KvLTQ1) forms cardiac-delayed rectifier-like K+ current in the heart with other subunits. Mutations in this channel can cause one form of inherited long QT syndrome (LQT1). KCNQ2 and KCNQ3 heteromultimers are thought to underlie the M-current; mutations in these genes may cause an inherited form of juvenile epilepsy. The KCNQ4 gene is thought to encode the molecular correlate of the 7K.n in outer hair cells of the cochlea mutations whose mutation leads to a form of inherited deafness. KCNQ5 co-assemble with KCNQ3 in brain, and may also play a role in the M-current heterogeneity.