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Ubiquitin-mediated mitochondrial regulation by MITOL/MARCHF5 at a glance

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JOURNAL OF BIOCHEMISTRY
卷 173, 期 1, 页码 1-11

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OXFORD UNIV PRESS
DOI: 10.1093/jb/mvac092

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ubiquitin; MITOL; MARCHF5; mitochondrial dynamics; mitochondria-ER contacts; mitochondria

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Mitochondria play a role in various cellular processes, and mitochondrial dysfunction is associated with age-related diseases. Mitochondrial quality is maintained through mitochondrial dynamics and the endoplasmic reticulum. MITOL controls mitochondrial quality through regulating mitochondrial dynamics, mitochondria-ER contacts, and mitophagy.
Mitochondria are involved in various cellular processes, such as energy production, inflammatory responses and cell death. Mitochondrial dysfunction is associated with many age-related diseases, including neurological disorders and heart failure. Mitochondrial quality is strictly maintained by mitochondrial dynamics linked to an adequate supply of phospholipids and other substances from the endoplasmic reticulum (ER). The outer mitochondrial membrane-localized E3 ubiquitin ligase MITOL/MARCHF5 is responsible for mitochondrial quality control through the regulation of mitochondrial dynamics, formation of mitochondria-ER contacts and mitophagy. MITOL deficiency has been shown to impair mitochondrial function, cause an excessive inflammatory response and increase vulnerability to stress, resulting in the exacerbation of the disease. In this study, we overview the ubiquitin-mediated regulation of mitochondrial function by MITOL and the relationship between MITOL and diseases.

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