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

作者：Ikeda Kazuhiro; Shiba Sachiko; Horie Inoue Kuniko; Shimokata Kunitoshi; Inoue Satoshi^*

来源：Nature Communications, 2013, 4(1): 2147.

DOI：10.1038/ncomms3147

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.

• 出版日期2013-7