兰尼碱受体是心肌细胞上的一种钙离子释放通道,当基因发生突变时可导致延迟后除极致心脏猝死性室性心动过速的发生,但到目前为止,具体的分子生物学机制却不是很清楚,可能与其调节蛋白-FK506结合蛋白的解离、Ca~(2+)激活域值降低及通道内部各功能域之间作用受损等引起的舒张期钙离子渗漏有关,现就关于基因突变所致兰尼碱受体功能障碍发生机制的各种学说做一综述。 Raney-base receptor is a kind of calcium channel in cardiomyocytes. When the gene mutation occurs, it can lead to the delayed onset of sudden cardiac sudden ventricular tachycardia. But until now, the specific molecular biological mechanism However, it is not clear that diastolic calcium leakage may be related to the dissociation of the regulatory protein-FK506-binding protein, the decrease of the activation domain of Ca 2+, and the impaired role of various functional domains within the channel. This review summarizes various theories about the mechanism of the occurrence of dysfunction of Ranitidine induced by gene mutation.