Article
Mutations in the gene encoding C8orf38 block complex I assembly by inhibiting production of the mitochondria-encoded subunit ND1.
Journal of molecular biology - 2 Dec 2011
McKenzie Matthew, Tucker Elena J, Compton Alison G, Lazarou Michael, George Christa, Thorburn David R, Ryan Michael T
Abstract excerpt
The assembly of complex I (NADH-ubiquinone oxidoreductase) is a complicated process, requiring the integration of 45 subunits encoded by both nuclear and mitochondrial DNAs into a structure of approximately 1 MDa. A number of "assembly factors" that aid complex I biogenesis have recently been described, including C8orf38. This protein was identified as an assembly factor by its evolutionary conservation in...
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