MTFMT formylates methionyl-tRNA

Stable Identifier
R-HSA-5389841
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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Like bacteria, mitochondria initiate translation with N-formylmethionine. Unlike bacteria, mammalian mitochondria do not have a tRNA dedicated to N-formylmethionine. Instead, the mitochondrial enzyme MTFMT (methionyl-tRNA formyltransferase, FMT, FMT1) transfers a formyl group from 10-formyltetrahydrofolate (10-formyl-THF) to the amino group of methionyl-tRNA in a portion of the methionyl-tRNAs in the matrix.
Literature References
PubMed ID Title Journal Year
21907147 Mutations in MTFMT underlie a human disorder of formylation causing impaired mitochondrial translation

Calvo, SE, Belcher-Timme, CA, Köhrer, C, Patel, J, Thorburn, DR, RajBhandary, UL, Mootha, VK, Jaffe, JD, McKenzie, M, Carr, SA, Compton, AG, Goldberger, OA, Ryan, MT, Silberstein, JM, Hershman, SG, Tucker, EJ, Christodoulou, J

Cell Metab. 2011
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Catalyst Activity

methionyl-tRNA formyltransferase activity of MTFMT [mitochondrial matrix]

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