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

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 Katunuma, N, Ohura, T, Kominami, E, Tada, K	J Biochem	1982
	The Metabolic and Molecular Bases of Inherited Disease, 8th ed Beaudet, AL, Scriver, CR, Sly, WS, Valle, D		2001
9514741	Crystal structure of human recombinant ornithine aminotransferase. Schirmer, T, Hohenester, E, Jansonius, JN, Shen, BW, Hennig, M	J Mol Biol	1998
1737786	Ornithine delta-aminotransferase mutations in gyrate atrophy. Allelic heterogeneity and functional consequences. Obie, C, Valle, D, Kaiser-Kupfer, M, Robert, MF, Sipila, I, Mitchell, GA, Steel, G, Michaud, J, Fontaine, G, Brody, LC	J Biol Chem	1992