ornithine + alpha-ketoglutarate <=> glutamate + L-glutamate gamma-semialdehyde [OAT]

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial ornithine aminotransferase (OAT) catalyzes the reversible reaction of ornithine and alpha-ketoglutarate to form glutamate semialdehyde and glutamate (Ohura et al. 1982). The active enzyme is a hexamer (Shen et al. 1998). Inherited OAT deficiency leads to ornithine accumulation in vivo and gyrate atrophy of the choroid and retina (Brody et al. 1992; Valle and Simell 2001).
Literature References
PubMed ID Title Journal Year
6819292 Crystallization and properties of human liver ornithine aminotransferase

Katunuma, N, Ohura, T, Kominami, E, Tada, K

J Biochem 1982
  The Metabolic and Molecular Bases of Inherited Disease, 8th ed

Beaudet, AL, Scriver, CR, Sly, WS, Valle, D

9514741 Crystal structure of human recombinant ornithine aminotransferase.

Schirmer, T, Hohenester, E, Jansonius, JN, Shen, BW, Hennig, M

J Mol Biol 1998
1737786 Ornithine delta-aminotransferase mutations in gyrate atrophy. Allelic heterogeneity and functional consequences.

Obie, C, Valle, D, Kaiser-Kupfer, M, Robert, MF, Sipila, I, Mitchell, GA, Steel, G, Michaud, J, Fontaine, G, Brody, LC

J Biol Chem 1992
Event Information
Catalyst Activity

ornithine aminotransferase activity of OAT hexamer [mitochondrial matrix]

Orthologous Events
Cross References
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