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电压门控钠离子通道Nav 1.5负责动作电位0期的快速除极以及冲动在工作心肌细胞间的传导,钠通道复合物及相关调节蛋白的异常可导致心律失常综合征。遗传性心律失常相关钠通道复合物蛋白有:心脏电压门控钠离子通道β1~β4亚单位;小窝蛋白3、三磷酸甘油醇脱氢酶-1类似物、MOG1和α-1互养蛋白。其它钠通道复合物相关蛋白有Ankyrin-G、β4Spectrin、SAP-97、钙调素、钙调素依赖的蛋白激酶Ⅱ、Plakophillin 2、Desmoglein 2、缝隙连接蛋白40和43等18种,这类蛋白能够调控钠离子电流,它们中的部分已在心律失常性心肌病中被发现存在突变,但在遗传性心律失常患者中还未被发现存在突变。
The voltage-gated sodium channel Nav 1.5 is responsible for the rapid depolarization of the action potential phase 0 and the conduction of impulses among the working cardiomyocytes. Abnormalities in sodium channel complexes and related regulatory proteins can lead to arrhythmia syndrome. Hereditary arrhythmia-related sodium channel complex protein: cardiac voltage-gated sodium channel β1 ~ β4 subunit; caveolin 3, triphosphate dehydrogenase-1 analogs, MOG1 and α-1 messenger protein . Other sodium channel complex-related proteins are Ankyrin-G, β4Spectrin, SAP-97, calmodulin, calmodulin-dependent protein kinase Ⅱ, Plakophillin 2, Desmoglein 2, connexin 40 and 43, 18 kinds of such proteins Can regulate sodium currents, some of them have been found to have mutations in arrhythmic cardiomyopathy, but no mutations have been found in patients with inherited arrhythmias.