Homolog of yeast mitochondrial ATP-dependent tRNA sulfurtransferase Mtu1. Sulfur group originates from cysteine via a cascade of sulphur relay proteins, including the pyridoxal phosphate-containing cysteine desulfurase Nfs1 and at least a third mitochondrial sulfur transport protein. In mitochondria of T. brucei (Eukarya), this third protein partner is Isd11.
Human TRMU encoding the mitochondrial 5-methylaminomethyl-2-thiouridylate-methyltransferase is a putative nuclear modifier gene for the phenotypic expression of the deafness-associated 12S rRNA mutations.
Yan Q, Bykhovskaya Y, Li R, Mengesha E, Shohat M, Estivill X, Fischel-Ghodsian N, Guan MX
Mitochondria-specific RNA-modifying enzymes responsible for the biosynthesis of the wobble base in mitochondrial tRNAs. Implications for the molecular pathogenesis of human mitochondrial diseases.
Umeda N, Suzuki T, Yukawa M, Ohya Y, Shindo H, Watanabe K, Suzuki T
Human mitochondrial diseases caused by lack of taurine modification in mitochondrial tRNAs.
Human mitochondrial tRNAs: biogenesis, function, structural aspects, and diseases.
Human TRMU encoding the mitochondrial 5-methylaminomethyl-2-thiouridylate-methyltransferase is a putative nuclear modifier gene for the phenotypic expression of the deafness-associated 12S rRNA mutations.
Mitochondria-specific RNA-modifying enzymes responsible for the biosynthesis of the wobble base in mitochondrial tRNAs. Implications for the molecular pathogenesis of human mitochondrial diseases.
The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation.