A stabilizing factor for mitochondrial respiratory supercomplex assembly regulates energy metabolism in muscle
Open accessAbstract
The mitochondrial respiratory chain is essential for oxidative phosphorylation and comprises multiple complexes, including cytochrome c oxidase, assembled in macromolecular supercomplexes. Little is known about factors that contribute to supercomplex organization. Here we identify COX7RP as a factor that promotes supercomplex assembly. Cox7rp-knockout mice exhibit decreased muscular activity and heat production failure in the cold due to reduced COX activity. In contrast, COX7RP-transgenic mice exhibit increased exercise performance with increased cytochrome c oxidase activity. Two-dimensional blue native electrophoresis reveals that COX7RP is a key molecule that promotes assembly of the III2/IVn supercomplex with complex I. Our study identified COX7RP as a protein that functions in I/III2/IVn supercomplex assembly and is required for full activity of mitochondrial respiration.
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Many mitochondrial proteins must be assembled into large, multi-subunit structures, some of which form supercomplexes that are essential for mitochondrial energy metabolism.
“many must be assembled into large, multi-subunit structures, some of which form “supercomplexes” [39] that are essential for mitochondrial energy metabolism [40]”
From Neuronal Apolipoprotein E4 Expression Results in Proteome-Wide Alterations and Compromises Bioenergetic Capacity by Disrupting Mitochondrial Function (2019) · cites this paper as [40]
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- Authors
- Ikeda, K., Shiba, S., Horie-Inoue, K., Shimokata, K., & Inoue, S.
- Affiliations
- Saitama Medical University (JP)
- The University of Tokyo (JP)
- Corresponding author
- Inoue, Satoshi
- Venue
- Nature Communications
- Published
- 2013-07-16
- Volume
- 4
- Issue
- 1
- Pages
- 2147-2147
- ISSN
- 2041-1723 (ISSN-L)
- OpenAlex
- W2033781650
- Cited by
- 152
- References
- 1
- Citations by year
- 2026: 14 · 2025: 5 · 2024: 9 · 2023: 15 · 2022: 8 · 2021: 8 · 2020: 14 · 2019: 8 · 2018: 12 · 2017: 17 · 2016: 15 · 2015: 12 · 2014: 10 · 2013: 5
- FWCI
- 5.27
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Nature Communications (publishedVersion)
- PubMed (publishedVersion)
- RePEc: Research Papers in Economics (submittedVersion)
- Funders
- Ministry of Education, Culture, Sports, Science and Technology
- Ministry of Health, Labour and Welfare
- Japan Society for the Promotion of Science (23249040)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Health Sciences › Medicine › Physiology › Adipose Tissue and Metabolism
- Keywords
- cytochrome c oxidase, complex I, oxidative phosphorylation, muscle energy metabolism, exercise performance, thermogenesis, cold tolerance, mitochondrial respiration, transgenic mice
- MeSH terms
- Animals, Body Temperature Regulation, Electron Transport Complex IV, Female, Gene Expression Regulation, Male, Mice, Transgenic, Mitochondria, Muscle, Oxidative Phosphorylation, Electron Transport Complex III, Muscle, Skeletal, Electron Transport Complex I, Mice
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