Full name: Mitochondrial tRNA-specific 2-thiouridylase 1
Synonym: Mto2, SLM3
GI: 6320172
Orf: D2761
COG: COG0482
UniProt: Q12093
Structures: | |
Complex:
Enzyme type: sulfurtransferase
Position of modification - modification: t:34 - cmnm5s2U

Comments:

Homolog of bacterial ATP-dependent tRNA sulphur transferase MnmA (alias TrmU). Sulfur group originates from cysteine via a cascade of sulphur relay proteins, of which the pyridoxal phosphate-containing cysteine desulfurase Nfs1 plays the same role as IscS in bacteria. At least one other mitochondrial sulfur transport protein remains to be identified (2012). U34 thiolation in mitochondria differs from that in bacteria, which involves IscS (instead of Nfs1) and a cascade of relay proteins TusA,B,C,D,E.

Protein sequence:

MLARYLNLIGRRSASPYRPQRLPAKFDNVIVAMSSGVDSSVAAALFAGEFPNTRGVYMQNWSESQSLDDP GKEPCYERDWRDVNRVAKHLNIRVDKVNFEQDYWIDVFEPMLRGYSEGSTPNPDIGCNKFVKFGKLREWL DEKYGTGNYWLVTGHYARVMQEMNGKGLFHLLRSIYRPKDQSYYLSQINSTVLSSLLLPIGHLTKPEVRD LAKYAGLPTAEKPDSQGICFVNNSQHGKFKNFLKHYLPSSPGDIITVDPQSGAKTTWGRHDGLWSYTIGQ KVGISMPQADPNYQGTWFVSEKLRDTNEILIVRGRDNPALYSDTMRIENFSSLGPREDTINAFQNTGALT LQFRSLQVPVQIKSCKLNRSADNLDITIHLASKQRAITPGQSCCLYIDDRVLGSGPISHVNNNDTHA

Enzymatic activities:

Reaction Substrate Type Position
cmnm5U:cmnm5s2U tRNA (t)   34
U:s2U tRNA (t) 34
nm5U:nm5s2U tRNA (t) 34
mnm5U:mnm5s2U tRNA (t) 34

Publications:

Title Authors Journal Details PubMed Id DOI
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 -
Deletion of the MTO2 gene related to tRNA modification causes a failure in mitochondrial RNA metabolism in the yeast Saccharomyces cerevisiae. Wang X, Yan Q, Guan MX FEBS Lett [details] 17706197 -
Mutations in MTO2 related to tRNA modification impair mitochondrial gene expression and protein synthesis in the presence of a paromomycin resistance mutation in mitochondrial 15 S rRNA. Yan Q, Li X, Faye G, Guan MX J Biol Chem [details] 15944150 -

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