【摘要】 线粒体是一种处于高度运动状态的细胞器,频繁地出现分裂和融合,线粒体分裂和融合的动态过程被称为线粒体动力学。对于神经元来说,线粒体的动力学过程具有十分重要的生物学意义。已知线粒体融合介导蛋白的功能缺失性突变可以导致常染色体显性遗传性视神经萎缩和Charcot-Marie-Tooth病等神经变性疾病。近来发现,在迟发性神经变性疾病中,线粒体动力学的改变也具有重要地位。本文将在线粒体动力学的分子调控以及与细胞死亡的关系、在神经变性疾病中的地位等方面综述这一领域的最新进展。更多还原

【Abstract】 Mitochondria are highly dynamic organelles,which undergo continuous cycles of fission and fusion.Mitochondrial dynamics(fission and fusion) plays important roles in maintaining neuronal functions,including biogenesis,mitochondrial distribution and cell injury or death.Mfn1/2 and Opa1 mediate mitochondrial fusion,whereas Drp1 and Fis1 regulate mitochondrial fission.Mutations of Opa1 cause autosomal dominant optic atrophy,and mutations of Mfn2 lead to Charcot-Marie-Tooth disease type 2A.Moreover,increasing evidences show that abnormal mitochondrial dynamics are involved in pathogenesis of late-onset neurodegenerative disorders,such as Alzheimer’s disease,Parkinson’s disease and Huntington’s disease.This paper reviews the current advances of abnormal mitochondrial dynamics relevant to neuronal loss in neurodegenerative diseases.更多还原