鸟氨酸转氨酶，一种在多种代谢途径交叉点上的重要谷氨酸代谢酶。
Ornithine Aminotransferase, an Important Glutamate-Metabolizing Enzyme at the Crossroads of Multiple Metabolic Pathways.
作者信息
Ginguay Antonin, Cynober Luc, Curis Emmanuel, Nicolis Ioannis
机构信息
Clinical Chemistry, Cochin Hospital, GH HUPC, AP-HP, 75014 Paris, France.
Laboratory of Biological Nutrition, EA 4466 PRETRAM, Faculté de Pharmacie, Université Paris Descartes, 75006 Paris, France.
出版信息
Biology (Basel). 2017 Mar 7;6(1):18. doi: 10.3390/biology6010018.
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.
鸟氨酸δ-氨基转移酶（OAT，酶编号E.C. 2.6.1.13）催化鸟氨酸（Orn）的δ-氨基转移至α-酮戊二酸（aKG），生成谷氨酸-5-半醛和谷氨酸（Glu），反之亦然。在哺乳动物中，OAT是一种线粒体酶，主要位于肝脏、肠道、大脑和肾脏。一般来说，OAT用于由鸟氨酸生成谷氨酸，但肠道是个显著例外，在肠道中瓜氨酸（Cit）或精氨酸（Arg）是终产物。其主要功能是控制信号分子和介质的产生，如Glu本身、Cit、γ-氨基丁酸（GABA）和脂肪族多胺。它还参与脯氨酸（Pro）的合成。OAT缺乏会导致回旋状萎缩，这是一种罕见但严重的遗传性疾病，进一步说明了这种酶的重要性。
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