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

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

Stable Identifier

R-HSA-70654

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

2-oxoglutarate + L-ornithine => L-glutamate + L-glutamate 5-semialdehyde

ReviewStatus

5/5

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

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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
6819292	Crystallization and properties of human liver ornithine aminotransferase Ohura, T, Kominami, E, Tada, K, Katunuma, N	J Biochem	1982
	The Metabolic and Molecular Bases of Inherited Disease, 8th ed Scriver, CR, Beaudet, AL, Valle, D, Sly, WS		2001
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