Reaction: glutamate + L-glutamate gamma-semialdehyde <=> ornithine + alpha-ketoglutarate [OAT]
- in pathway: Glutamate and glutamine metabolism
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).
Reaction - small molecule participants:
2OG [mitochondrial matrix]
L-Orn [mitochondrial matrix]
L-Glu5S [mitochondrial matrix]
L-Glu [mitochondrial matrix]
Reactome.org reaction link: R-HSA-70666
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Reaction input - small molecules:
L-glutamic 5-semialdehyde zwitterion
L-glutamate(1-)
Reaction output - small molecules:
2-oxoglutarate(2-)
L-ornithinium(1+)
Reactome.org link: R-HSA-70666