Protein SummaryTrmU from Homo sapiens |
| Full name: | Mitochondrial tRNA-specific 2-thiouridylase 1 |
|---|---|
| Synonym: | MTU1, TRMT1 |
| GI: | 31542641 |
| Orf: | |
| COG: | COG0482 |
| UniProt: | O75648 |
| Complex: | |
| Enzyme type: | sulfurtransferase |
| Position of modification - modification: |
t:34 - tm5s2U |
Comments:
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.Protein sequence:
|
MQALRHVVCALSGGVDSAVAALLLRRRGYQVTGVFMKNWDSLDEHGVCTA DKDCEDAYRVCQILDIPFHQVSYVKEYWNDVFSDFLNEYEKGRTPNPDIV CNKHIKFSCFFHYAVDNLGADAIATGHYARTSLEDEEVFEQKHVKKPEGL FRNRFEVRNAVKLLQAADSFKDQTFFLSQVSQDALRRTIFPLGGLTKEFV KKIAAENRLHHVLQKKESMGMCFIGKRNFEHFLLQYLQPRPGHFISIEDN KVLGTHKGWFLYTLGQRANIGGLREPWYVVEKDSVKGDVFVAPRTDHPAL YRDLLRTSRVHWIAEEPPAALVRDKMMECHFRFRHQMALVPCVLTLNQDG TVWVTAVQAVRALATGQFAVFYKGDECLGSGKILRLGPSAYTLQKGQRRA GMATESPSDSPEDGPGLSPLL |
Enzymatic activities
| Reaction | Substrate | Type | Position |
|---|---|---|---|
| U:s2U | tRNA (t) | 34 | |
| cmnm5U:cmnm5s2U | tRNA (t) | 34 | |
| nm5U:nm5s2U | tRNA (t) | 34 | |
| mnm5U:mnm5s2U | tRNA (t) | 34 | |
| mcm5U:mcm5s2U | tRNA (t) | 34 | |
| tm5U:tm5s2U | tRNA (t) | Lys/∃UU/mitochondrial | 34 |
| m5U:m5s2U | tRNA (t) | 34 | |
| Um:s2Um | tRNA (t) | 34 |
Publications
| 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 | - |
Links
| _PubMed_ |
Last modification of this entry: 2012-11-30 15:02:15.853051
Edited by a user: magda
Edited content: Changed papers.
