Protein Summary

TrmU 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 PubMed Id DOI
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.