Article
An N-terminal formyl methionine on COX 1 is required for the assembly of cytochrome c oxidase.
Human molecular genetics - 15 Jul 2015
Hinttala Reetta, Sasarman Florin, Nishimura Tamiko, Antonicka Hana, Brunel-Guitton Catherine, Schwartzentruber Jeremy, Fahiminiya Somayyeh, Majewski Jacek, Faubert Denis, Ostergaard Elsebet, Smeitink Jan A, Shoubridge Eric A
Abstract excerpt
Protein synthesis in mitochondria is initiated by formylmethionyl-tRNA(Met) (fMet-tRNA(Met)), which requires the activity of the enzyme MTFMT to formylate the methionyl group. We investigated the molecular consequences of mutations in MTFMT in patients with Leigh syndrome or cardiomyopathy. All p...
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