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

Stable Identifier
R-HSA-70654
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
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
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
Participants
Participates
Catalyst Activity

ornithine-oxo-acid transaminase activity of OAT hexamer [mitochondrial matrix]

Orthologous Events
Cross References
Rhea
Cite Us!