Pourrier Marc, Nattel Stanley
Département de pharmacologie et Centre de recherche, Université de Montréal, Québec, Canada.
Med Sci (Paris). 2004 Apr;20(4):437-41. doi: 10.1051/medsci/2004204437.
Mutations in proteins responsible for ion transport in cardiac tissue can induce a destabilization of electrical function and provoke cardiac sudden death. Identification of a genetic anomaly in a French family that developed the syndrome of cardiac sudden death has revealed a crucial new element in normal cardiac electrical function : Ion channels need to be anchored to specific domains at the plasma membrane by an anchoring protein called ankyrin-B.
负责心脏组织中离子转运的蛋白质发生突变,可能会导致电功能不稳定,引发心源性猝死。对一个出现心源性猝死综合征的法国家庭进行基因异常鉴定,揭示了正常心脏电功能中一个关键的新因素:离子通道需要通过一种名为锚蛋白B的锚定蛋白锚定在质膜的特定区域。