GOT2 transaminates OA and L-Glu

Stable Identifier
R-HSA-70613
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
GOT2 transaminates oxaloacetate and glutamate, L-glutamate + oxaloacetate => 2-oxoglutarate + L-aspartate
ReviewStatus
5/5
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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
31422819 Bi-allelic GOT2 Mutations Cause a Treatable Malate-Aspartate Shuttle-Related Encephalopathy

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

Am J Hum Genet 2019
26902786 Recombinant expression, purification and crystallographic studies of the mature form of human mitochondrial aspartate aminotransferase

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

Biosci Trends 2016
4052435 The primary structure of mitochondrial aspartate aminotransferase from human heart

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

Biochim Biophys Acta 1985
Participants
Participates
Catalyst Activity

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

Orthologous Events
Cross References
Rhea
RHEA
Reviewed
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