Canine Ventricular KCNE2 Expression Resides Predominantly in Purkinje Fibers
Canine Ventricular KCNE2 Expression Resides Predominantly in Purkinje Fibers
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DOI：
10.1161/01.res.0000084851.60947.b5
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发表时间：
2003-08
期刊：
影响因子：
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通讯作者：
M. Pourrier;S. Zicha;J. Ehrlich;W. Han;S. Nattel
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文献类型：
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作者：
M. Pourrier;S. Zicha;J. Ehrlich;W. Han;S. Nattel
minK相关肽1（MiRP 1）突变，KCNE 2基因的产物，已与恶性室性心律失常综合征相关的受损复极。MiRP 1与多种离子通道亚基相互作用，其功能障碍可能导致心律失常综合征;然而，观察到心室组织中MiRP 1的极低水平表达导致对其相关性的怀疑。特化的希氏-浦肯野系统在心脏电生理学中起关键作用，并且是与异常复极相关的室性心律失常的重要促成因素。我们研究了犬浦肯野组织与心室组织中MiRP 1的相对丰度，发现浦肯野组织中mRNA和蛋白质水平的表达要高得多。因此，心脏浦肯野系统是一个强有力的候选人发挥作用，由于MiRP 1异常的糖尿病综合征。
Mutations in minK-related peptide 1 (MiRP1), the product of the KCNE2 gene, have been associated with malignant ventricular arrhythmia syndromes related to impaired repolarization. MiRP1 interacts with a variety of ion-channel -subunits, dysfunction of which could account for arrhythmia syndromes; however, the observation of very low-level expression of MiRP1 in ventricular tissue has led to doubts about its relevance. The specialized His-Purkinje system plays a key role in cardiac electrophysiology and is an important contributor to ventricular arrhythmias related to abnormal repolarization. We examined the relative abundance of MiRP1 in canine Purkinje versus ventricular tissue and found much greater expression at both mRNA and protein levels in Purkinje tissue. Thus, the cardiac Purkinje system is a strong candidate to play a role in arrhythmic syndromes due to MiRP1 abnormalities.