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

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial ornithine aminotransferase (OAT) catalyzes the reversible reaction of glutamate semialdehyde and glutamate to form ornithine and alpha-ketoglutarate (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; Vallee and Simell 2001).

Literature References
PubMed ID Title Journal Year
1737786 Ornithine delta-aminotransferase mutations in gyrate atrophy. Allelic heterogeneity and functional consequences.

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

J Biol Chem 1992
  The Metabolic and Molecular Bases of Inherited Disease, 8th ed

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

Participant Of
Event Information
Catalyst Activity
Catalyst Activity
ornithine-oxo-acid transaminase activity of OAT hexamer [mitochondrial matrix]
Physical Entity
Orthologous Events