Article
Mutations in MTFMT underlie a human disorder of formylation causing impaired mitochondrial translation.
Cell metabolism - 7 Sept 2011
Tucker Elena J, Hershman Steven G, Köhrer Caroline, Belcher-Timme Casey A, Patel Jinal, Goldberger Olga A, Christodoulou John, Silberstein Jonathon M, McKenzie Matthew, Ryan Michael T, Compton Alison G, Jaffe Jacob D, Carr Steven A, Calvo Sarah E, RajBhandary Uttam L, Thorburn David R, Mootha Vamsi K
Abstract excerpt
The metazoan mitochondrial translation machinery is unusual in having a single tRNA(Met) that fulfills the dual role of the initiator and elongator tRNA(Met). A portion of the Met-tRNA(Met) pool is formylated by mitochondrial methionyl-tRNA formyltransferase (MTFMT) to generate N-formylmethionine-tRNA(Met) (fMet-tRNA(met)), which is used for translation initiation; however, the requirement of formylation for...
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