GOT2 transaminates OA and L-Glu

Stable Identifier
R-HSA-70613
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
L-glutamate + oxaloacetate => 2-oxoglutarate + L-aspartate, GOT2 transaminates oxaloacetate and glutamate
ReviewStatus
5/5
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GOT2 transaminates OA and L-Glu
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Mitochondrial GOT2 (glutamate oxaloacetate transaminase 2, also known as aspartate aminotransferase, mitochondrial) catalyzes the reversible transamination of oxaloacetate (OA) and glutamate (L-Glu) to form aspartate (L-Asp) and 2-oxoglutarate (2-OG, alpha-ketoglutarate) (Martini et al. 1985). The active form of the enzyme is a homodimer of GOT2 monomers each conjugated with pyridoxal phosphate on lysine residue 279 (Jiang et al., 2016). Mutations in MDH1 can cause a form of early-onset epileptic encephalopathy (DEE82, MIM:618721; van Karnebeek et al. 2019).
Literature References
PubMed ID Title Journal Year
4052435 The primary structure of mitochondrial aspartate aminotransferase from human heart

Martini, F, Angelaccio, S, Barra, D, Pascarella, S, Maras, B, Doonan, S, Bossa, F

Biochim Biophys Acta 1985
26902786 Recombinant expression, purification and crystallographic studies of the mature form of human mitochondrial aspartate aminotransferase

Jiang, X, Wang, J, Chang, H, Zhou, Y

Biosci Trends 2016
31422819 Bi-allelic GOT2 Mutations Cause a Treatable Malate-Aspartate Shuttle-Related Encephalopathy

van Karnebeek, CDM, Ramos, RJ, Wen, XY, Tarailo-Graovac, M, Gleeson, JG, Skrypnyk, C, Brand-Arzamendi, K, Karbassi, F, Issa, MY, van der Lee, R, Drögemöller, BI, Koster, J, Rousseau, J, Campeau, PM, Wang, Y, Cao, F, Li, M, Ruiter, J, Ciapaite, J, Kluijtmans, LAJ, Willemsen, MAAP, Jans, JJ, Ross, CJ, Wintjes, LT, Rodenburg, RJ, Huigen, MCDG, Jia, Z, Waterham, HR, Wasserman, WW, Wanders, RJA, Verhoeven-Duif, NM, Zaki, MS, Wevers, RA

Am J Hum Genet 2019
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Catalyst Activity

L-aspartate:2-oxoglutarate aminotransferase activity of GOT2 dimer [mitochondrial matrix]

Physical Entity
GOT2 dimer [mitochondrial matrix] (Homo sapiens)
Activity
L-aspartate:2-oxoglutarate aminotransferase activity (GO:0004069)
Orthologous Events
GOT2 transaminates OA and L-Glu (Bos taurus)
GOT2 transaminates OA and L-Glu (Caenorhabditis elegans)
GOT2 transaminates OA and L-Glu (Canis familiaris)
GOT2 transaminates OA and L-Glu (Danio rerio)
GOT2 transaminates OA and L-Glu (Dictyostelium discoideum)
GOT2 transaminates OA and L-Glu (Drosophila melanogaster)
GOT2 transaminates OA and L-Glu (Gallus gallus)
GOT2 transaminates OA and L-Glu (Mus musculus)
GOT2 transaminates OA and L-Glu (Plasmodium falciparum)
GOT2 transaminates OA and L-Glu (Rattus norvegicus)
GOT2 transaminates OA and L-Glu (Saccharomyces cerevisiae)
GOT2 transaminates OA and L-Glu (Schizosaccharomyces pombe)
GOT2 transaminates OA and L-Glu (Sus scrofa)
Cross References
RHEA
Reviewed
Harris, RA  (2008-09-10)
Hill, DP  (2022-08-29)
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