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

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

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

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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