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Biology (Basel). 2017 Mar 7;6(1). pii: E18. doi: 10.3390/biology6010018.

Ornithine Aminotransferase, an Important Glutamate-Metabolizing Enzyme at the Crossroads of Multiple Metabolic Pathways.

Ginguay A1,2, Cynober L3,4, Curis E5,6,7,8, Nicolis I9,10.

Author information

1
Clinical Chemistry, Cochin Hospital, GH HUPC, AP-HP, 75014 Paris, France. antonin.ginguay@gmail.com.
2
Laboratory of Biological Nutrition, EA 4466 PRETRAM, Faculté de Pharmacie, Université Paris Descartes, 75006 Paris, France. antonin.ginguay@gmail.com.
3
Clinical Chemistry, Cochin Hospital, GH HUPC, AP-HP, 75014 Paris, France. luc.cynober@parisdescartes.fr.
4
Laboratory of Biological Nutrition, EA 4466 PRETRAM, Faculté de Pharmacie, Université Paris Descartes, 75006 Paris, France. luc.cynober@parisdescartes.fr.
5
Laboratoire de biomathématiques, plateau iB², Faculté de Pharmacie, Université Paris Descartes, 75006 Paris, France. emmanuel.curis@parisdescartes.fr.
6
UMR 1144, INSERM, Université Paris Descartes, 75006 Paris, France. emmanuel.curis@parisdescartes.fr.
7
UMR 1144, Université Paris Descartes, 75006 Paris, France. emmanuel.curis@parisdescartes.fr.
8
Service de biostatistiques et d'informatique médicales, hôpital Saint-Louis, Assistance publique-hôpitaux de Paris, 75010 Paris, France. emmanuel.curis@parisdescartes.fr.
9
Laboratoire de biomathématiques, plateau iB², Faculté de Pharmacie, Université Paris Descartes, 75006 Paris, France. ioannis.nicolis@parisdescartes.fr.
10
EA 4064 "Épidémiologie environnementale: Impact sanitaire des pollutions", Faculté de Pharmacie, Université Paris Descartes, 75006 Paris, France. ioannis.nicolis@parisdescartes.fr.

Abstract

Ornithine δ-aminotransferase (OAT, E.C. 2.6.1.13) catalyzes the transfer of the δ-amino group from ornithine (Orn) to α-ketoglutarate (aKG), yielding glutamate-5-semialdehyde and glutamate (Glu), and vice versa. In mammals, OAT is a mitochondrial enzyme, mainly located in the liver, intestine, brain, and kidney. In general, OAT serves to form glutamate from ornithine, with the notable exception of the intestine, where citrulline (Cit) or arginine (Arg) are end products. Its main function is to control the production of signaling molecules and mediators, such as Glu itself, Cit, GABA, and aliphatic polyamines. It is also involved in proline (Pro) synthesis. Deficiency in OAT causes gyrate atrophy, a rare but serious inherited disease, a further measure of the importance of this enzyme.

KEYWORDS:

glutamate; gyrate atrophy; ornithine; ornithine aminotransferase

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