N-Carbamyl-L-glutamic acid (carglumic acid) is use to study its potential as a replacement therapy for N-acetylglutamate synthetase (NAGS) deficiency and to treat hyperammonemia.
Molecular genetics and metabolism, 79(4), 303-304 (2003-09-02)
In patients with methylmalonic aciduria (MMA), the accumulating metabolite propiony-CoA results in an inhibition of the urea circle via the decreased synthesis of N-acetylglutamate, an essential activator of carbamylphosphat synthetase (CPS). This results in one of the major clinical problems
The objectives of this study were to investigate the potential mechanisms of dietary arginine (Arg) and N-carbamoylglutamate (NCG) supplementation on reproductive performance of sows. Twenty-seven crossbred (Landrace×Large White) sows with similar body weight and parity at day (90±1) of gestation
Journal of animal science, 90 Suppl 4, 337-339 (2013-02-13)
To evaluate the effects of glutamate (Glu) or combination of Glu and N-carbamylglutamate (NCG) on intestinal mucosa morphology and epithelium cell proliferation, 18 piglets weaned at 21 d (BW 5.56 ± 0.51 kg) were grouped into 3 treatments and fed
The Journal of pediatrics, 145(4), 552-554 (2004-10-14)
In a patient with N-acetylglutamate synthase (NAGS) deficiency, incorporation of an isotopic label from ammonium chloride into urea was markedly reduced before treatment with N-carbamyl-L-glutamate (NCLG) and completely normalized following treatment. Blood ammonia rose following ammonium tracer ingestion before treatment
The Journal of pediatrics, 147(2), 260-262 (2005-08-30)
In a prospective study, patients with a suspected urea cycle defect underwent oral N-carbamoyl-L-glutamic acid loading testing. In patients with subsequently confirmed N-acetylglutamate synthase deficiency, hyperammonemia normalized within 8 hours. This test may be useful in the early diagnosis of
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