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Ornithine aminotransferase (OAT) is a mitochondrial enzyme that catalyzes the reversible transfer of the δ-amino group from L-ornithine to α-ketoglutarate, producing glutamate-5-semialdehyde and glutamate. It plays a key role in the interconversion of ornithine, proline, and glutamate, linking the urea cycle with proline and glutamate metabolism. Deficiency leads to gyrate atrophy, a rare inherited disorder.
Gabaculine: Inhibits OAT by covalently binding to the PLP cofactor site.
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