窖蛋白与不明原因心源性猝死的相关性研究进展

摘要

不明原因心源性猝死(unexpected sudden cardiac death,USCD)因其不伴有心脏结构的异常,尸体解剖呈阴性改变,一直是法医病理学鉴定的热点难题.USCD可能与部分致死性心律失常有关,该类心律失常多由心脏离子通道蛋白或其相关蛋白发生异常所致.窖蛋白可以通过其脚手架区域与多种心肌离子通道蛋白结合,在维持心肌动作电位的去极化和复极化中起到关键作用.当窖蛋白由于基因突变或蛋白表达异常等因素导致其结构和功能受到影响时,受其调控的心肌离子通道的功能也受到损害,继而引起多种离子通道病的发生,出现心律失常甚至心源性猝死.研究窖蛋白对离子通道功能的影响对于探索恶性心律失常及心源性猝死的发生机制具有重要意义.%D ue to the negative autopsy and w ithout cardiac structural abnorm alities, unexpected sudden cardiac death (U SC D ) is alw ays a tough issue for forensic pathological expertise. U SC D m ay be asso-ciated w ith parts of fatal arrhythm ic diseases. T hese arrhythm ic diseases m ay be caused by disorders of cardiac ion channels or channel-related proteins. C aveolin can com bine w ith m ultiple m yocardial ion channel proteins through its scaffolding regions and plays an im portant role in m aintaining the depolar-ization and repolarization of cardiac action potential. W hen the structure and function of caveolin are af-fected by gene m utations or abnorm al protein expression, the functions of the regulated ion channels are correspondingly im paired, w hich leads to the occurrence of m ultiple channelopathies, arrhythm ia or even sudden cardiac death. It is im portant to study the effects of caveolin on the functions of ion channels for exploring the m echanism s of m alignant arrhythm ia and sudden cardiac death.