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