OPA1的研究进展

OPA1（Optic Atrophy 1）基因属于核基因,编码的蛋白是线粒体内源发动蛋白,是线粒体塑形蛋白家族的成员。OPA1蛋白通过不同位点的剪接,形成多种亚型,参与线粒体内膜融合,对线粒体形态结构有着重要的作用。OPA1与呼吸作用复合物直接相关,作为呼吸链的一部分,保持呼吸链的完整性,参与呼吸作用和能量代谢;在细胞凋亡过程中则以OPA1-PARL复合体的形式发挥抗凋亡因子的作用。研究显示,OPA1在类固醇物质的生成等方面,也有着不可替代的作用。OPA1对多种疾病有影响,是显性视神经萎缩症（Dominant Optic Atrophy,DOA）的主要基因座,OPA1突变不仅会导致视觉疾病,也能引起听觉神经病变.OPA1还参与热休克应答,在抗癌药毒性抑制方面也有重要作用。本文着重于介绍OPA1的结构与功能,及其在疾病中的作用。

Research Progress of OPA1

OPA1 encoded by a nuclear gene,belongs to mitochondria shaping protein family,which is a mitochondrial inner membrane GTPase dynamic.Its variants generated by cheavaging in diffrent positions involve in mitochondrial inner membrane fusion,and play important role in mitochondrial structure.OPA1 is directly related with four respiration complexes and affects mitochondrial respiration.In apoptosis,it plays a role as anti-apoptotic factor.Recent research shows it has function in steroidogenesis and mitochondrial respiration.OPA1 has effects on a variety of diseases,for example,Dominant Optic Atrophy.OPA1 mutants not only result in Optic disease,but also involve in Auditory neuropathy,furthermore,it work as a anti-apoptotic in heat shock response and toxicity of anticancer drugs suppression.The article focus on introducing the structure of OPA1 and its function in disease.