期刊
JOURNAL OF INTERNAL MEDICINE
卷 287, 期 6, 页码 645-664出版社
WILEY
DOI: 10.1111/joim.13031
关键词
cristae membranes; human disease; MICOS; mitochondria; mitochondriopathies; oxidative phosphorylation
资金
- Deutsche Forschungsgemeinschaft [PF 202/9-1]
- Excellence Initiative/Strategy of the German Federal and State Governments (EXC 2189 CIBSS) [390939984]
- Excellence Initiative/Strategy of the German Federal and State Governments (EXC 294 BIOSS)
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 F1Fo-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.
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