Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial

Details

Name
Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial
Synonyms
  • 6.3.5.-
  • Cytochrome c oxidase assembly factor PET112 homolog
  • Glu-AdT subunit B
  • PET112
  • PET112L
Gene Name
GATB
Organism
Humans
Amino acid sequence
>lcl|BSEQ0004391|Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial
MAAPMLRWGCRGRRWAFARVDGGSCHRRGAPTGSTSNQIRGESSVAQQPLHTAQKTRKGE
HKWAAVVGLEIHAQISSNSKLFSGSQVRFSAPPNSLVSFFDASLPGTLPVLNRRCVEAAV
MTGLALNCHINKKSLFDRKHYFYADLPAGYQITQQRLPIAVNGSLIYGVCAGKKQSQVIP
KTVRIKQIQLEQDSGKSLHDNLRSQTLIDLNRAGVGLLEVVLEPDMSCGEEAATAVRELQ
LILQALGTSQANMAEGQLRVDANISVHHPGEPLGVRTEVKNLNSIRFLAKAIDYEIQRQI
NELENGGEILNETRSFHHKLGCTMSMRDKEGKQDYRFMPEPNLPPLVLYDATSLPAGADP
QQVINIDQIRETLPELPSVTREKLVQQYGMLLEHSFTLLNEVGLLEFFQNVIKETRAEPK
KVTSWVLNTFLGYLKQQNLAVSESPVTPSALAELLDLLDSRTISSSAAKQVFEELWKREG
KTPGQIVSEKQLELMQDQGALEQLCHSVMEAHPQVVMDVKNRNPRAINKLIGLVRKATQS
RADPVMIKEILEKKLSL
Number of residues
557
Molecular Weight
61863.57
Theoretical pI
8.86
GO Classification
Functions
ATP binding / glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity / translation factor activity, RNA binding
Processes
glutaminyl-tRNAGln biosynthesis via transamidation / mitochondrial translation / translation
Components
glutamyl-tRNA(Gln) amidotransferase complex / mitochondrion
General Function
Translation factor activity, rna binding
Specific Function
Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln).
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Mitochondrion
Gene sequence
>lcl|BSEQ0019257|Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial (GATB)
ATGGCGGCGCCCATGCTGCGCTGGGGCTGCCGTGGAAGACGTTGGGCTTTCGCCCGGGTT
GACGGTGGTTCTTGCCACCGAAGAGGGGCTCCGACTGGGTCCACATCCAACCAGATTAGG
GGAGAGAGCTCAGTGGCTCAGCAGCCCCTCCACACGGCCCAGAAGACGAGGAAAGGTGAA
CACAAATGGGCTGCTGTGGTAGGTTTGGAAATTCATGCCCAGATTTCCTCCAACTCTAAA
CTCTTCTCTGGATCTCAAGTTCGCTTTTCAGCACCTCCAAATTCTTTGGTTTCTTTTTTT
GATGCATCTCTACCTGGAACTTTGCCGGTTCTCAACAGGAGGTGTGTAGAAGCGGCGGTG
ATGACAGGCCTGGCTCTGAACTGCCACATAAACAAGAAGTCCTTGTTTGACAGGAAGCAC
TACTTCTATGCAGACCTCCCTGCAGGCTACCAAATTACCCAGCAGAGGCTCCCAATTGCT
GTGAATGGGAGCTTGATATATGGCGTCTGTGCAGGGAAGAAGCAGAGTCAGGTGATCCCC
AAGACGGTGAGGATCAAGCAGATCCAGTTGGAGCAAGACAGTGGCAAAAGCCTCCACGAC
AACCTGAGGTCTCAGACGCTCATTGATTTGAACAGGGCAGGAGTGGGCCTTCTGGAGGTG
GTCCTGGAGCCCGACATGTCCTGTGGAGAAGAGGCGGCAACAGCTGTCAGGGAGCTGCAG
CTGATCCTTCAAGCCCTGGGGACCAGCCAGGCGAACATGGCAGAGGGCCAGTTGAGAGTG
GATGCCAATATATCCGTGCATCACCCTGGGGAGCCTTTGGGCGTTCGAACGGAAGTGAAG
AATCTCAACAGCATCAGGTTCCTGGCCAAAGCCATAGACTATGAAATTCAGAGGCAAATC
AATGAACTTGAGAATGGAGGTGAAATTCTGAACGAAACACGCTCATTTCATCACAAGCTG
GGGTGCACCATGTCAATGAGAGACAAAGAAGGAAAACAGGACTACAGGTTCATGCCAGAA
CCCAACCTGCCTCCCCTGGTGCTCTACGACGCCACATCTCTGCCCGCAGGTGCAGACCCA
CAGCAAGTGATCAATATTGACCAGATTCGGGAGACACTCCCGGAGCTCCCCAGTGTGACC
CGAGAGAAGCTTGTCCAACAGTATGGGATGCTGCTGGAACACAGCTTCACTTTGCTGAAC
GAAGTCGGCCTACTGGAGTTCTTCCAAAATGTGATAAAAGAAACTAGGGCAGAGCCAAAA
AAGGTGACTAGTTGGGTCCTCAACACTTTTCTGGGCTATTTAAAGCAACAGAACCTCGCT
GTCAGTGAGAGTCCTGTCACACCCTCTGCACTCGCTGAGCTTCTTGACCTGCTGGACAGC
AGAACAATTTCTTCATCAGCAGCTAAACAGGTGTTTGAGGAACTGTGGAAGAGGGAAGGC
AAGACTCCAGGGCAGATTGTTTCAGAAAAGCAGCTTGAACTGATGCAGGACCAGGGGGCA
CTGGAGCAGCTCTGCCACTCTGTGATGGAGGCCCATCCTCAAGTGGTAATGGATGTGAAG
AACAGAAACCCCAGAGCTATAAATAAACTGATTGGGTTGGTCCGGAAAGCGACTCAAAGC
CGAGCAGATCCAGTCATGATAAAGGAGATCCTGGAGAAGAAGCTGTCATTGTGA
Chromosome Location
4
Locus
4q27-q28
External Identifiers
ResourceLink
UniProtKB IDO75879
UniProtKB Entry NameGATB_HUMAN
GenBank Protein ID3599964
GenBank Gene IDAF026851
GenAtlas IDPET112L
HGNC IDHGNC:8849
General References
  1. Petruzzella V, Tiranti V, Fernandez P, Ianna P, Carrozzo R, Zeviani M: Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain. Genomics. 1998 Dec 15;54(3):494-504. [Article]
  2. Zhang QH, Ye M, Wu XY, Ren SX, Zhao M, Zhao CJ, Fu G, Shen Y, Fan HY, Lu G, Zhong M, Xu XR, Han ZG, Zhang JW, Tao J, Huang QH, Zhou J, Hu GX, Gu J, Chen SJ, Chen Z: Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells. Genome Res. 2000 Oct;10(10):1546-60. [Article]
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  5. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
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  7. Nagao A, Suzuki T, Katoh T, Sakaguchi Y, Suzuki T: Biogenesis of glutaminyl-mt tRNAGln in human mitochondria. Proc Natl Acad Sci U S A. 2009 Sep 22;106(38):16209-14. doi: 10.1073/pnas.0907602106. Epub 2009 Sep 9. [Article]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00130L-Glutamineapproved, investigational, nutraceuticalunknownsubstrateDetails