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Protein

Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial

Gene

GATB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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).UniRule annotation1 Publication

Catalytic activityi

ATP + L-glutamyl-tRNA(Gln) + L-glutamine = ADP + phosphate + L-glutaminyl-tRNA(Gln) + L-glutamate.UniRule annotation

GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity Source: UniProtKB
  • translation factor activity, RNA binding Source: ProtInc

GO - Biological processi

  • glutaminyl-tRNAGln biosynthesis via transamidation Source: UniProtKB
  • mitochondrial translation Source: UniProtKB
  • translation Source: ProtInc

Keywordsi

Molecular functionLigase
Biological processProtein biosynthesis
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDAi6.3.5.7 2681

Names & Taxonomyi

Protein namesi
Recommended name:
Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrialUniRule annotation (EC:6.3.5.-UniRule annotation)
Short name:
Glu-AdT subunit BUniRule annotation
Alternative name(s):
Cytochrome c oxidase assembly factor PET112 homolog
Gene namesi
Name:GATBUniRule annotationImported
Synonyms:PET112UniRule annotation, PET112LUniRule annotation
ORF Names:HSPC199
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

EuPathDBiHostDB:ENSG00000059691.11
HGNCiHGNC:8849 GATB
MIMi603645 gene
neXtProtiNX_O75879

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000059691
PharmGKBiPA33191

Chemistry databases

DrugBankiDB00142 L-Glutamic Acid
DB00130 L-Glutamine

Polymorphism and mutation databases

BioMutaiPET112

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 41MitochondrionSequence analysisAdd BLAST41
ChainiPRO_000001071042 – 557Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrialAdd BLAST516

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei529N6-succinyllysineBy similarity1

Proteomic databases

EPDiO75879
PaxDbiO75879
PeptideAtlasiO75879
PRIDEiO75879

PTM databases

iPTMnetiO75879
PhosphoSitePlusiO75879

Expressioni

Tissue specificityi

Predominantly expressed in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle and heart.1 Publication

Gene expression databases

BgeeiENSG00000059691
CleanExiHS_PET112L
ExpressionAtlasiO75879 baseline and differential
GenevisibleiO75879 HS

Organism-specific databases

HPAiHPA042610

Interactioni

Subunit structurei

Subunit of the heterotrimeric GatCAB amidotransferase (AdT) complex, composed of A (QRSL1), B (GATB) and C (GATC) subunits.

Protein-protein interaction databases

BioGridi111211, 12 interactors
DIPiDIP-48968N
IntActiO75879, 6 interactors
STRINGi9606.ENSP00000263985

Structurei

3D structure databases

ProteinModelPortaliO75879
SMRiO75879
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the GatB/GatE family. GatB subfamily.UniRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2438 Eukaryota
COG0064 LUCA
GeneTreeiENSGT00390000016644
HOGENOMiHOG000223743
HOVERGENiHBG003160
InParanoidiO75879
KOiK02434
OMAiSDGNMQE
OrthoDBiEOG091G099P
PhylomeDBiO75879
TreeFamiTF314355

Family and domain databases

Gene3Di1.10.10.410, 1 hit
HAMAPiMF_00121 GatB, 1 hit
InterProiView protein in InterPro
IPR004413 Apn/Gln-ADT_bsu
IPR017959 Asn/Gln-tRNA_amidoTrfase_suB/E
IPR006075 Asn/Gln-tRNA_Trfase_suB/E_cat
IPR018027 Asn/Gln_amidotransferase
IPR003789 Asn/Gln_tRNA_amidoTrase-B-like
IPR023168 GatB_Yqey_C
IPR017958 Gln-tRNA_amidoTrfase_suB_CS
IPR014746 Gln_synth/guanido_kin_cat_dom
PANTHERiPTHR11659 PTHR11659, 1 hit
PfamiView protein in Pfam
PF02934 GatB_N, 1 hit
PF02637 GatB_Yqey, 1 hit
SMARTiView protein in SMART
SM00845 GatB_Yqey, 1 hit
SUPFAMiSSF55931 SSF55931, 1 hit
SSF89095 SSF89095, 1 hit
TIGRFAMsiTIGR00133 gatB, 1 hit
PROSITEiView protein in PROSITE
PS01234 GATB, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O75879-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAAPMLRWGC RGRRWAFARV DGGSCHRRGA PTGSTSNQIR GESSVAQQPL
60 70 80 90 100
HTAQKTRKGE HKWAAVVGLE IHAQISSNSK LFSGSQVRFS APPNSLVSFF
110 120 130 140 150
DASLPGTLPV LNRRCVEAAV MTGLALNCHI NKKSLFDRKH YFYADLPAGY
160 170 180 190 200
QITQQRLPIA VNGSLIYGVC AGKKQSQVIP KTVRIKQIQL EQDSGKSLHD
210 220 230 240 250
NLRSQTLIDL NRAGVGLLEV VLEPDMSCGE EAATAVRELQ LILQALGTSQ
260 270 280 290 300
ANMAEGQLRV DANISVHHPG EPLGVRTEVK NLNSIRFLAK AIDYEIQRQI
310 320 330 340 350
NELENGGEIL NETRSFHHKL GCTMSMRDKE GKQDYRFMPE PNLPPLVLYD
360 370 380 390 400
ATSLPAGADP QQVINIDQIR ETLPELPSVT REKLVQQYGM LLEHSFTLLN
410 420 430 440 450
EVGLLEFFQN VIKETRAEPK KVTSWVLNTF LGYLKQQNLA VSESPVTPSA
460 470 480 490 500
LAELLDLLDS RTISSSAAKQ VFEELWKREG KTPGQIVSEK QLELMQDQGA
510 520 530 540 550
LEQLCHSVME AHPQVVMDVK NRNPRAINKL IGLVRKATQS RADPVMIKEI

LEKKLSL
Length:557
Mass (Da):61,864
Last modified:November 1, 1998 - v1
Checksum:i449EB3EA2FD4769D
GO

Sequence cautioni

The sequence AAF36119 differs from that shown. Reason: Frameshift at position 420.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti317H → R in BAD96607 (Ref. 4) Curated1
Sequence conflicti418 – 419EP → DK in AAF36119 (PubMed:11042152).Curated2
Sequence conflicti521N → D in BAD96607 (Ref. 4) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04912830A → D. Corresponds to variant dbSNP:rs11556167Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF026851 mRNA Translation: AAD08640.1
AF151033 mRNA Translation: AAF36119.1 Frameshift.
AK312957 mRNA Translation: BAG35796.1
AK222887 mRNA Translation: BAD96607.1
AC092611 Genomic DNA Translation: AAY40897.1
CH471056 Genomic DNA Translation: EAX04981.1
BC130348 mRNA Translation: AAI30349.1
BC136547 mRNA Translation: AAI36548.1
AB019410 mRNA No translation available.
CCDSiCCDS3776.1
RefSeqiNP_004555.1, NM_004564.2
UniGeneiHs.119316
Hs.636045

Genome annotation databases

EnsembliENST00000263985; ENSP00000263985; ENSG00000059691
GeneIDi5188
KEGGihsa:5188
UCSCiuc003iml.5 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiGATB_HUMAN
AccessioniPrimary (citable) accession number: O75879
Secondary accession number(s): Q4W5M8
, Q53GP4, Q9P0S6, Q9Y2B8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: November 1, 1998
Last modified: May 23, 2018
This is version 149 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

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