Abstract:
: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.
journal_name
Nat Communjournal_title
Nature communicationsauthors
Ikeda K,Shiba S,Horie-Inoue K,Shimokata K,Inoue Sdoi
10.1038/ncomms3147subject
Has Abstractpub_date
2013-01-01 00:00:00pages
2147issn
2041-1723pii
ncomms3147journal_volume
4pub_type
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