Methionyl-tRNA formyltransferase, mitochondrial (MtFMT, MTFMT, FMT, FMT1)

Function

• formylates methionyl-tRNA in mitochondria
• a single tRNA(Met) gene gives rise to both an initiator & an elongator species via an unknown mechanism

10-formyltetrahydrofolate + L-methionyl-tRNA(fMet) + H2O
                   <-->
tetrahydrofolate + N-formylmethionyl-tRNA(fMet)

Structure

• belongs to the fmt family
• N-terminal domain carries the tetrahydrofolate (THF)- binding site
• C-terminal domain is presumably involved in positioning the Met-tRNA substrate for formylation reaction

Compartment

mitochondria

Alternative splicing

named isoforms=2

Pathology

• defects associated with
  • combined oxidative phosphorylation deficiency 15
  • Leigh syndrome

Laboratory

• MTFMT gene mutation

More general terms

• mitochondrial protein
• transferase

Database

• UniProt: http://www.uniprot.org/uniprot/Q96DP5.html
• OMIM: https://mirror.omim.org/entry/614947
• OMIM: https://mirror.omim.org/entry/256000