A stabilizing factor for mitochondrial respiratory supercomplex assembly regulates energy metabolism in muscle

Kazuhiro Ikeda, Sachiko Shiba, Kuniko Horie-Inoue, Kunitoshi Shimokata, Satoshi Inoue
2013 Nature Communications  
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
more » ... 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 III 2 /IV n supercomplex with complex I. Our study identified COX7RP as a protein that functions in I/III 2 /IV n supercomplex assembly and is required for full activity of mitochondrial respiration.
doi:10.1038/ncomms3147 pmid:23857330 fatcat:omjjzvd7wba5bexhkqk7ytk5oi