Mitochondrial dysfunction underlying metabolic disorders such as obesity and diabetes mellitus is strongly associated with cardiac arrhythmias. Murine Pgc-1α^−/− hearts replicate disrupted mitochondrial function and model the associated pro-arrhythmic electrophysiological abnormalities. Quantitative PCR, western blotting and histological analysis were used to investigate the molecular basis of the electrophysiological changes associated with mitochondrial dysfunction. qPCR analysis implicated downregulation of genes related to Na⁺-K⁺ ATPase activity (Atp1b1), surface Ca²⁺ entry (Cacna1c), action potential repolarisation (Kcnn1), autonomic function (Adra1d, Adcy4, Pde4d, Prkar2a), and morphological properties (Myh6, Tbx3) in murine Pgc-1α^−/− ventricles. Western blotting revealed reduced Na_V1.5 but normal Cx43 expression. Histological analysis revealed increased tissue fibrosis in the Pgc-1α^−/− ventricles. These present findings identify altered transcription amongst a strategically selected set of genes established as encoding proteins involved in cardiac electrophysiological activation and therefore potentially involved in alterations in ventricular activation and Ca²⁺ homeostasis in arrhythmic substrate associated with Pgc-1α deficiency. They complement and complete previous studies examining such expression characteristics in the atria and ventricles of Pgc-1 deficient murine hearts.

肥胖和糖尿病等代谢性疾病潜在的线粒体功能障碍与心律失常密切相关。小鼠 Pgc-1α ^-/−心脏复制受损的线粒体功能并模拟相关的促心律失常电生理异常。使用定量PCR、蛋白质印迹和组织学分析来研究与线粒体功能障碍相关的电生理变化的分子基础。 qPCR 分析表明与 Na ⁺ -K ⁺ ATP 酶活性 (Atp1b1)、表面 Ca ²⁺进入 (Cacna1c)、动作电位复极化 (Kcnn1)、自主功能（Adra1d、Adcy4、Pde4d、Prkar2a）和形态特性相关的基因下调(Myh6, Tbx3) 在小鼠 Pgc-1α ^−/−心室中。 Western blotting 显示 Na _V 1.5 表达减少，但 Cx43 表达正常。组织学分析显示 Pgc-1α ^−/−心室组织纤维化增加。这些目前的发现确定了一组策略性选择的基因中转录的改变，这些基因被确定为编码参与心脏电生理激活的蛋白质，因此可能参与心室激活和与 Pgc-1α 缺乏相关的心律失常底物中 Ca ²⁺稳态的改变。他们补充并完成了之前检查 Pgc-1 缺陷小鼠心脏心房和心室中此类表达特征的研究。