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

Stable Identifier
R-HSA-70654
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
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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
  The Metabolic and Molecular Bases of Inherited Disease, 8th ed

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

  2001
6819292 Crystallization and properties of human liver ornithine aminotransferase

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

J Biochem 1982
9514741 Crystal structure of human recombinant ornithine aminotransferase.

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

J Mol Biol 1998
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
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