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SCN5A-△KPQ变异的长QT综合征的细胞电生理 被引量：1

The study of cellular mechanism(s) underlying ventricular-arrhythmogenesis of △KPQ LQT_3
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摘要 目的探讨长QT综合征3型(LQT3)的致病基因SCN5A及其相关的变异产生室性心律失常的细胞电生理机制。方法从野生型小鼠和SCN5A+/△的小鼠心脏中分离心室肌细胞,采用电流嵌和全细胞膜片钳技术分析了携带SCN5A△KPQ变异的小鼠心肌细胞的电生理学表型。结果与野生型细胞[(55.0±6.6)ms,n=7]相比,SCN5A+/△心肌细胞[(152±17.8)ms,n=6]动作电位明显延长(P<0.01);在SCN5A+/△心肌细胞中观察到早期后除极,而野生型心肌细胞中则未出现;全细胞记录表明SCN5A+/△心肌细胞有晚期持续钠电流,而在野生型细胞中未见。结论晚期持续钠电流可能是与SCN5A△KPQ突变相关的室性心律失常的主要机制。 Objective:To understand cellular mechanism(s) underlying ventricular-arrhythmogenesis of DKPQ LQT_3. Method:The electrophysiological phenotype was studied in isolated adult ventricular myocytes from mice harbouring SCN5A-△KPQ mutation for LQT_3. Result: SCN5A-△KPQ myocytes showed a significant prolongation of action potential (AP) duration (152±18 ms, n=6) compared to wild-type myocytes (55±6.6 ms, n=7,P<~0.01 ) and early-afterdepolarization like action potential was also observed in some of SCN5A-△KPQ myocytes. Conclusion:These findings suggest that the late current could be a major mechanism responsible for the ventricular arrhythmias associated with this mutation.
作者 雷鸣 Goddard C 孙思 Colledge W H Grace A A Huang C L
机构地区 华中科技大学协和医院心内科华中科技大学同济医学院心血管病研究所 Cardiovascular Group 华中科技大学协和医院血液科华中科技大学同济医学院干细胞中心
出处 《临床心血管病杂志》 CAS CSCD 北大核心 2005年第4期195-197,共3页 Journal of Clinical Cardiology
基金 国家自然科学基金资助(No:30470634)
关键词 长QT综合征 SCN5A基因 全细胞膜片钳技术 电生理学研究 心脏 LQT3 SCN5A gene Whole cell patch clamp Electrophysiologic study, cardiac
分类号 R442.8 [医药卫生—诊断学]
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参考文献6

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同被引文献18

  • 1Rodriguez-Calvo MS, Brion M, Allegue C, et al. Molecular genetics of sudden cardiac death. Forensic Sci Int,2008,182 : 1 - 12.
  • 2Fatkin D, MacRae C, Sasaki T, et al. Missense mutations in the rod domain of the lamin A/C gene as causes of dilated eardiomyopathy and conduction-system disease. N Engl J Med, 1999, 341 : 1715-1724.
  • 3Syrris P, Murray A, Carter ND, et al. Mutation detection in long QT syndrome: a comprehensive set of primers and PCR conditions. J Med Genet,2001,38 :705-710.
  • 4Goldenberg I, Zareba W, Moss AJ. Long QT Syndrome. Curr Probl Cardiol,2008, 33:629-694.
  • 5Itoh T, Tanka T, Ryozo N, et al. Genomic organization and mutational analysis of KCNH2, a gene responsible for familial long QT syndrome. Hum Genet, 1998,102 : 435-439.
  • 6Zareba W, Cygankiewicz I. Long QT Syndrome and Short QT Syndrome. Prog Cardiovasc Dis,2008,51:264-278.
  • 7Morita H, Wu J, Zipes DP. The QT syndromes: long and short. Lancet ,2008,372 : 750-763.
  • 8Splawski I, Shen J, Timothy KW, et al. Spectrum of mutations in long-QT syndrome genes: KVLQT1, HERG, SCNSA, KCNE1, and KCNE2. Circulation, 2000,102 : 1178-1185.
  • 9Zimmer T, Surber R. SCNSA channelopathies: An update on mutations and mechanisms. Prog Biophys Mol Biol, 2008,5 : 1- 17.
  • 10Moss A J, Kass RS. Long QT syndrome: from channels to cardiac arrhythmias. J Clin Invest, 2005,115 : 2018-2024.

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临床心血管病杂志
2005年 第4期

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