| First Author | Ikeda K | Year | 2013 |
| Journal | Nat Commun | Volume | 4 |
| Pages | 2147 | PubMed ID | 23857330 |
| Mgi Jnum | J:215964 | Mgi Id | MGI:5607399 |
| Doi | 10.1038/ncomms3147 | Citation | Ikeda K, et al. (2013) A stabilizing factor for mitochondrial respiratory supercomplex assembly regulates energy metabolism in muscle. Nat Commun 4:2147 |
| abstractText | 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. |
