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A salt or ester of glutamic acid.
g. acetyltransferase an enzyme catalyzing transfer of an acetyl group from N2-acetylornithine to l-g. forming l-ornithine and N-acetyl-l-glutamate, an activator of the urea cycle.ornithine acetyltransferase;
g. decarboxylase (GAD) a carboxy-lyase converting l-g. to 4-aminobutyrate and CO2 as well as l-aspartate to 3-aminopropanoate and CO2; a defect in the binding of this protein's coenzyme is believed to be the cause of pyridoxine dependency with seizures.aspartate 1-decarboxylase;
g. dehydrogenases enzymes that catalyze the reaction of l-g., H2O, and NAD+ (or NADP+ in some cases) producing a-ketoglutarate (2-oxoglutarate), ammonia, and NADH; in mammals, this is the prime contributor to oxidative deamination.glutamic acid dehydrogenases;
g. formiminotransferase an enzyme that catalyzes the transfer of the formimino moiety of N-formimino-l-glutamate to tetrahydrofolate; a deficiency of this enzyme will lead to elevated formiminoglutamate levels.
g. gamma-semialdehyde -OOCCH(NH3)+CH2CH2CHO;an intermediate in l-proline and l-ornithine metabolism; becomes elevated in type II hyperprolinemia.
g. synthase an enzyme that converts l-glutamine, a-ketoglutarate, and NADH (in some cases, NADPH) to two l-g.'s and NAD+ (or, NADP+); apparently, a nonmammalian enzyme.
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