MTFMT formylates methionyl-tRNA

Stable Identifier
R-HSA-5389841
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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

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

Cell Metab. 2011
Participants
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Catalyst Activity
Title
methionyl-tRNA formyltransferase activity of MTFMT [mitochondrial matrix]
Physical Entity
Activity
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