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

Stable Identifier
R-HSA-70666
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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

  2001
Participants
Participant Of
Event Information
Catalyst Activity
Catalyst Activity
Title
ornithine-oxo-acid transaminase activity of OAT hexamer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea