Full name: | Mitochondrial tRNA-specific 2-thiouridylase 1 |
---|---|
Synonym: | MTU1, TRMT1 |
GI: | 31542641 |
COG: | COG0482 |
UniProt: | O75648 |
Alpha Fold Predicted Structure: | AF-O75648-F1 |
Enzyme type: | sulfurtransferase |
Position of modification - modification: |
t:34 - tm5s2U |
MQALRHVVCALSGGVDSAVAALLLRRRGYQVTGVFMKNWDSLDEHGVCTADKDCEDAYRVCQILDIPFHQVSYVKEYWNDVFSDFLNEYEKGRTPNPDIVCNKHIKFSCFFHYAVDNLGADAIATGHYARTSLEDEEVFEQKHVKKPEGLFRNRFEVRNAVKLLQAADSFKDQTFFLSQVSQDALRRTIFPLGGLTKEFVKKIAAENRLHHVLQKKESMGMCFIGKRNFEHFLLQYLQPRPGHFISIEDNKVLGTHKGWFLYTLGQRANIGGLREPWYVVEKDSVKGDVFVAPRTDHPALYRDLLRTSRVHWIAEEPPAALVRDKMMECHFRFRHQMALVPCVLTLNQDGTVWVTAVQAVRALATGQFAVFYKGDECLGSGKILRLGPSAYTLQKGQRRAGMATESPSDSPEDGPGLSPLL
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.
Reaction | Substrate | SubstrateType | Position | (Anti)Codon | Modified (Anti)Codon | Amino Acid Change | Transcript Name | Transcript Region | Cellular Localization | References |
---|---|---|---|---|---|---|---|---|---|---|
U:s2U | RNA | tRNA | 34 | UUU | 2UU | tRNALysUUU | wobble - position | Mitochondrion | 15509579    | |
cmnm5U:cmnm5s2U | RNA | tRNA | 34 | !UU | $UU | tRNALys!UU | wobble - position | Mitochondrion | 15509579    | |
nm5U:nm5s2U | RNA | tRNA | 34 | ∪UU | ∫UU | tRNALys∪UU | wobble - position | Mitochondrion | 15509579    | |
mnm5U:mnm5s2U | RNA | tRNA | 34 | {UU | SUU | tRNALys{UU | wobble - position | Mitochondrion | 15509579    | |
mcm5U:mcm5s2U | RNA | tRNA | 34 | {UU | 3UU | tRNALys{UU | wobble - position | Mitochondrion | 15509579    | |
tm5U:tm5s2U | RNA | tRNA | 34 | ÊUU | ∃UU | tRNALysGUA | wobble - position | Mitochondrion | 15509579    | |
m5U:m5s2U | RNA | tRNA | 34 | TUU | 3UU | tRNALysTUU | wobble - position | Mitochondrion | 15509579    | |
Um:s2Um | RNA | tRNA | 34 | JUU | ∏UU | tRNALysJUU | wobble - position | Mitochondrion |
Alpha Fold Pdb Files | AF-O75648-F1.pdb   |
Alpha Fold Pdbx/mmCIF Files | AF-O75648-F1.cif   |
DSSP Secondary Structures | O75648.dssp   |
Title | Authors | Journal | Details | ||
---|---|---|---|---|---|
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 | Biochem Biophys Res Commun | [details] | 16513084 | - |
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 | J Biol Chem | [details] | 15509579 | - |
Human mitochondrial tRNAs: biogenesis, function, structural aspects, and diseases. | Suzuki T, Nagao A, Suzuki T | Annu Rev Genet | [details] | 21910628 | - |
Human mitochondrial diseases caused by lack of taurine modification in mitochondrial tRNAs. | Suzuki T, Nagao A, Suzuki T | Wiley Interdiscip Rev RNA | [details] | 21957023 | - |
The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation. | Sasarman F, Antonicka H, Horvath R, Shoubridge EA | Hum Mol Genet | [details] | 21890497 | - |
_PubMed_ |