Shaping the mitochondrial inner membrane in health and disease.,Journal of Internal Medicine

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Shaping the mitochondrial inner membrane in health and disease.
Journal of Internal Medicine ( IF 9.0 ) Pub Date : 2020-02-03 , DOI: 10.1111/joim.13031
L Colina-Tenorio _{1,

2} , P Horten _{1,

3} , N Pfanner _{1,

2,

4} , H Rampelt _{1,

2}

Affiliation

Mitochondria play central roles in cellular energetics, metabolism and signalling. Efficient respiration, mitochondrial quality control, apoptosis and inheritance of mitochondrial DNA depend on the proper architecture of the mitochondrial membranes and a dynamic remodelling of inner membrane cristae. Defects in mitochondrial architecture can result in severe human diseases affecting predominantly the nervous system and the heart. Inner membrane morphology is generated and maintained in particular by the mitochondrial contact site and cristae organizing system (MICOS), the F1 Fo -ATP synthase, the fusion protein OPA1/Mgm1 and the nonbilayer-forming phospholipids cardiolipin and phosphatidylethanolamine. These protein complexes and phospholipids are embedded in a network of functional interactions. They communicate with each other and additional factors, enabling them to balance different aspects of cristae biogenesis and to dynamically remodel the inner mitochondrial membrane. Genetic alterations disturbing these membrane-shaping factors can lead to human pathologies including fatal encephalopathy, dominant optic atrophy, Leigh syndrome, Parkinson's disease and Barth syndrome.

中文翻译：

在健康和疾病中塑造线粒体内膜。

线粒体在细胞能量，代谢和信号传导中起着核心作用。有效的呼吸，线粒体质量控制，线粒体DNA的凋亡和遗传取决于线粒体膜的适当结构以及内膜cr的动态重塑。线粒体结构缺陷可能导致严重的人类疾病，主要影响神经系统和心脏。内膜形态特别是通过线粒体接触位点和cr组织系统（MICOS），F1 Fo -ATP合酶，融合蛋白OPA1 / Mgm1和非双层磷脂质心磷脂和磷脂酰乙醇胺生成和维持的。这些蛋白质复合物和磷脂被嵌入功能相互作用的网络中。它们彼此之间以及其他因素之间进行交流，使它们能够平衡of生物发生的不同方面，并动态地重塑线粒体内膜。遗传改变扰乱了这些膜形成因子，可导致人类疾病，包括致命性脑病，显性视神经萎缩，利氏综合征，帕金森氏病和巴特综合征。

更新日期：2020-02-03

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