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

Stable Identifier
R-HSA-70666
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
L-glutamate + L-glutamate 5-semialdehyde => 2-oxoglutarate + L-ornithine
ReviewStatus
5/5
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glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT]
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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
  The Metabolic and Molecular Bases of Inherited Disease, 8th ed

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

  2001
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
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Output
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as an event of
Event Information
Reverse Reaction
ornithine + alpha-ketoglutarate <=> glutamate + L-glutamate gamma-semialdehyde [OAT] (Homo sapiens)
Catalyst Activity

ornithine aminotransferase activity of OAT hexamer [mitochondrial matrix]

Physical Entity
OAT hexamer [mitochondrial matrix] (Homo sapiens)
Activity
ornithine aminotransferase activity (GO:0004587)
Orthologous Events
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Bos taurus)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Caenorhabditis elegans)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Canis familiaris)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Danio rerio)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Dictyostelium discoideum)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Drosophila melanogaster)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Gallus gallus)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Mus musculus)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Plasmodium falciparum)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Rattus norvegicus)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Saccharomyces cerevisiae)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Schizosaccharomyces pombe)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Sus scrofa)
glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT] (Xenopus tropicalis)
Cross References
RHEA
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