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KCNQ钾通道研究进展 被引量：1

Research progress in KCNQ potassium channels
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摘要 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。 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.
作者 张炜 王晓良
机构地区 中国医学科学院.协和医料大学药物研究所
出处 《中国药理学通报》 CAS CSCD 北大核心 2003年第6期605-609,共5页 Chinese Pharmacological Bulletin
关键词 KCNQ通道 LQT综合征 M电流 癫痫 KCNQ channels LQT syndrome M-current epilepsy
分类号 R-05 [医药卫生] R329.24 [医药卫生—人体解剖和组织胚胎学]
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参考文献20

  • 1[1]Wang Q, Curran ME, Splawski I et al. Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias. Nat Genet, 1996;12(1):17～23
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同被引文献27

  • 1Wang Q,Curran M E,Splawski I,et al. Positional cloning of a novel potassium channel gene:KVLQT1 mutations cause cardiac arrhythmias[ J]. Nat Genet, 1996,12( 1 ) :17 -23.
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  • 6Melman Y F, Um S Y, Krumerman A,et al. KCNE1 binds to the KCNQ1 pore to regulate potassium channel activity [ J ]. Neuron, 2004,42:927 - 37.
  • 7Tinel N,Diochot S, Borsotto M,et al. KCNE2 confers background current characteristics to the cardiac KCNQ1 potassium channel [J]. EMBO J,2000,19:6326 -30.
  • 8Schroeder B C, Waldegger S, Fehr S, et al. A constitutively open potassium channel formed by KCNQ1 and KCNE3 [ J]. Nature, 2000,403 : 196 - 9.
  • 9Grunnet M,Jespersen T,Rasmussen H B,et al. KCNE4 is an inhibitory subunit to the KCNQ1 channel [ J ]. J Physiol, 2002,542 : 119 -30.
  • 10Piccini M, Vitelli F, Seri M,et al. KCNEI-like gene is deleted in AMME contiguous gene syndrome : identification and characterization of the human and mouse homologs [ J ]. Genomics, 1999,60 : 251 -7.

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中国药理学通报
2003年 第6期

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