PDF(Pubmed)
Abstract：
Nav1.5 is the predominant cardiac sodium channel subtype, encoded by the SCN5A gene, which is involved in the initiation and conduction of action potentials throughout the heart. Along its biosynthesis process, Nav1.5 undergoes strict genomic and non-genomic regulatory and quality control steps that allow only newly synthesized channels to reach their final membrane destination and carry out their electrophysiological role. These regulatory pathways are ensured by distinct interacting proteins that accompany the nascent Nav1.5 protein along with different subcellular organelles. Defects on a large number of these pathways have a tremendous impact on Nav1.5 functionality and are thus intimately linked to cardiac arrhythmias. In the present review, we provide current state-of-the-art information on the molecular events that regulate SCN5A/Nav1.5 and the cardiac channelopathies associated with defects in these pathways.
摘要：
Nav1.5是主要的心脏钠通道亚型，由SCN5A基因编码，它参与动作电位在整个心脏的启动和传导。在它的生物合成过程中，Nav1.5经过严格的基因组和非基因组调节和质量控制步骤，仅允许新合成的通道到达其最终的膜目的地并发挥其电生理作用。这些调节途径由伴随新生的Nav1.5蛋白以及不同亚细胞器的不同相互作用蛋白确保。大量这些途径的缺陷对Nav1.5功能具有巨大影响，因此与心律失常密切相关。在本次审查中，我们提供了有关调节SCN5A/Nav1.5的分子事件以及与这些通路缺陷相关的心脏通道病变的最新信息.
