N-acetylglutamate

N-a·ce·tyl·glu·ta·mate (NAG),

(a-sĕ'til-glū'tă-māt),
The salt of N-acetylglutamic acid. An activator of carbamoyl phosphate synthetase I during urea synthesis; this amino acid causes a configurational change in the enzyme, thus increasing its activity. The inability to synthesize N-acetylglutamate results in a defect in urea biosynthesis.
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Keywords: N-Carbamoylglutamte, N-Acetylglutamate, Rumen fluid, Ion chromatograph Determination, Method
N-carbamoylglutamate (NCG) is a feed additives obtained synthetically from sodium glutamate, which is a structural analogue of N-acetylglutamate [1], that is an essential factor of carbamoyl phosphate synthetase-1, a rate-liming enzyme that augments urea cycle and the arginine (AGR) production process [2] and have favorable position of lower rumen breakdown compared to ARG [3].
Raviciti is not indicated for treatment of acute hyperammonemia in patients with UCDs, and its safety and efficacy for the treatment of n-acetylglutamate synthase deficiency has not been established.
New insights on the mechanisms of valproate-induced hyperammonemia: Inhibition of hepatic N-acetylglutamate synthase activity by valproyl-CoA.
Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency.
With [absolute value of [P.sub.corr]] values > 0.60207 (for degree of freedom = 9) and VIP values > 1, the urine samples of the MS group compared to those of the NS group showed upregulation of leucine, lactate, alanine, dimethylamine, phenylacetylglycine, hippurate, allantoin, cytidine, tyrosine, imidazole, and phenylalanine and downregulation of acetate, N-acetylglutamate, succinate, 2-oxoglutarate, citrate, and trimethylamine (Table 1).
According to the contract, Medunik Canada will receive Canadian rights to market and distribute four important therapies in acute hepatic porphyria, hyperammonaema due to N-acetylglutamate synthase deficiency or one of three organic acidurias, patent ductus arteriosus and vitamin E deficiency in chronic cholestasis.
The three endocrine products are carglumic acid (Carbaglu; C) for treatment of hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS); tesamorelin (Egrifta; X) a growth hormone--releasing factor analog for reduction of excess abdominal fat in HIV-infected patients; and velaglucerase alfa (VPRIV; B) given for long-term enzyme replacement in patients with Gaucher disease.
The urea cycle consists of 6 nuclear genome-encoded enzymes, 3 of which are located in the mitochondrial matrix: CPS1 [7] (carbamoyl-phosphate synthetase 1, mitochondrial), NAGS (N-acetylglutamate synthase), and OTC (ornithine carbamoyltransferase).
The US Food and Drug Administration (FDA) has approved the New Drug Application (NDA) submitted by Orphan Europe for the use of Carbaglu in paediatric and adult patients for the treatment of acute hyperammonaemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS deficiency) and as maintenance therapy for chronic hyperammonaemia due to NAGS deficiency.
The hyperammonemia observed in methylmalonic acidemia is thought to arise because accumulated propionyl CoA interferes with formation of N-acetylglutamate, an obligatory activator of carbamyl phosphate synthase, the initial step in urea synthesis (4).

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