Mitochondrial methionyl-tRNA formyltransferase (MTFMT)

Mitochondrial methionyl-tRNA formyltransferase (MTFMT) is an enzyme encoded by the nuclear MTFMT gene that localizes to mitochondria, where it catalyzes the formylation of methionyl-tRNA (Met-tRNA), a key step required for translation initiation in mitochondria[2][4][5]. This activity parallels the initiation seen in bacteria, as mammalian mitochondria use N-formylmethionine to start protein synthesis. Mutations in MTFMT significantly reduce mitochondrial translation efficiency and are associated with mitochondrial diseases such as Leigh syndrome and combined oxidative phosphorylation deficiency, due to defective synthesis of mitochondrial proteins necessary for respiratory chain function[1][2][6].

Other names

Methionyl-tRNA formyltransferase, mitochondrialMTFMT

Inhibition or modulation of MTFMT could affect mitochondrial translation and energy metabolism (inference based on function)[1][5].