Entity Details

Primary name GATB_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionO75879
EntryNameGATB_HUMAN
FullNameGlutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length557
SequenceStatuscomplete
DateCreated2000-05-30
DateModified2021-06-02

Ontological Relatives

GenesGATB

GO terms

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GOName
GO:0005524 ATP binding
GO:0005739 mitochondrion
GO:0030956 glutamyl-tRNA(Gln) amidotransferase complex
GO:0032543 mitochondrial translation
GO:0050567 glutaminyl-tRNA synthase (glutamine-hydrolyzing) activity
GO:0070681 glutaminyl-tRNAGln biosynthesis via transamidation

Subcellular Location

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Subcellular Location
Mitochondrion

Domains

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DomainNameCategoryType
IPR003789 Aspartyl/glutamyl-tRNA amidotransferase subunit B-likeFamilyHomologous superfamily
IPR004413 Aspartyl/glutamyl-tRNA(Asn/Gln) amidotransferase, B subunitFamilyFamily
IPR006075 Aspartyl/Glutamyl-tRNA(Gln) amidotransferase, subunit B/E, catalyticDomainDomain
IPR014746 Glutamine synthetase/guanido kinase, catalytic domainFamilyHomologous superfamily
IPR017958 Glutamyl-tRNA(Gln) amidotransferase, subunit B, conserved siteSiteConserved site
IPR017959 Aspartyl/glutamyl-tRNA(Asn/Gln) amidotransferase, subunit B /EFamilyFamily
IPR018027 Asn/Gln amidotransferaseDomainDomain
IPR023168 Aspartyl/glutamyl-tRNA(Asn/Gln) amidotransferase, C-terminal, domain 2FamilyHomologous superfamily
IPR042114 Aspartyl/glutamyl-tRNA(Asn/Gln) amidotransferase, C-terminal, domain 1FamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
618838 OMIMCombined oxidative phosphorylation deficiency 41 (COXPD41)An autosomal recessive mitochondrial disorder characterized by prenatal onset, fetal hydrops, intrauterine growth retardation, hypertrophic cardiomyopathy, respiratory insufficiency, lactic acidosis, and decreased activities of mitochondrial respiratory complexes I, III, IV, and V. The disorder is lethal, with death occurring in the perinatal period. The disease may be caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00130 L-GlutamineDrugbanksmall molecule

Interactions

3 interactions