Reactome | GOT2 transaminates oxaloacetate and glutamate

GOT2 transaminates oxaloacetate and glutamate

Stable Identifier

R-HSA-70613

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

L-glutamate + oxaloacetate => 2-oxoglutarate + L-aspartate

ReviewStatus

5/5

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GOT2 transaminates oxaloacetate and glutamate

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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; van Karnebeek et al. 2019). The active form of the enzyme is a homodimer of GOT2 monomers each conjugated with pyridoxal phosphate on lysine residue 279.

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
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