. . . "Humans and other mammals"@en . . . "Carbamino-L-glutamic acid"@en . "Carglumic acid is an orphan drug used for the treatment of hyperammonaemia in patients with N-acetylglutamate synthase deficiency. This rare genetic disorder results in elevated blood levels of ammonia, which can eventually cross the blood\u2013brain barrier and cause neurologic problems, cerebral edema, coma, and death. Carglumic acid was approved by the U.S. Food and Drug Administration (FDA) on 18 March 2010."@en . . "N-Carbamoyl-L-Glutamic Acid"@en . . . . . "(S)-2-ureidopentanedioic acid"@en . . "Carglumic acid is a synthetic structural analogue of N-acetylglutamate (NAG), which is an essential allosteric activator of the liver enzyme carbamoyl phosphate synthetase 1 (CPS1). CPS1 is found in the mitochondria and is the first enzyme of the urea cycle, which converts ammonia into urea. Carglumic acid acts as a replacement for NAG in NAGS deficiency patients by activating CPS1 but it does not help to regulate the urea cycle."@en . "N-Carbamyl-L-glutamate"@en . . . "1188-38-1"@en . . "(2S)-2-(Carbamoylamino)pentanedioic acid"@en . . . "Carbamylglutamic acid"@en . "N-Carbamylglutamate"@en . . . . . . "The apparent total clearance was 5.7 L/min (range 3.0-9.7), the renal clearance was 290 mL/min (range 204-445), and the 24-hour urinary excretion was 4.5 % of the dose (range 3.5-7.5)."@en . . . . "Carglumic Acid"@en . "Acido carglumico"@en . . "LD50, oral, mouse: >1000 mg/kg "@en . . "The apparent volume of distribution was 2657 L (range: 1616-5797)."@en . . . "# Elpeleg O, Shaag A, Ben-Shalom E, Schmid T, Bachmann C: N-acetylglutamate synthase deficiency and the treatment of hyperammonemic encephalopathy. Ann Neurol. 2002 Dec;52(6):845-9. \"Pubmed\":http://www.ncbi.nlm.nih.gov/pubmed/12447942 # Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M: Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. J Pediatr. 2004 Oct;145(4):552-4. \"Pubmed\":http://www.ncbi.nlm.nih.gov/pubmed/15480384 # Summary of the European Public Assessment Report (EPAR) from European Medicines Agency. Last updated on 21/09/2009. \"Carbaglu\":http://www.ema.europa.eu/ema/index.jsp?curl=pages/medicines/human/medicines/000461/human_med_000685.jsp&murl=menus/medicines/medicines.jsp&mid=WC0b01ac058001d124&jsenabled=true # Thompson CA: Carglumic acid approved to treat genetic hyperammonemia. Am J Health Syst Pharm. 2010 May 1;67(9):690. \"Pubmed\":http://www.ncbi.nlm.nih.gov/pubmed/20410539 # Haberle J: Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency. Ther Clin Risk Manag. 2011;7:327-32. doi: 10.2147/TCRM.S12703. Epub 2011 Aug 2. \"Pubmed\":http://www.ncbi.nlm.nih.gov/pubmed/21941437"@en . "Carbaglu"@en . . "Acidum carglumicum"@en . . . . . . . "approved"@en . "For the treatment of acute and chronic hyperammonaemia in patients with N-acetylglutamate synthase (NAGS) deficiency. This enzyme is an important component of the urea cycle to prevent build up of neurotoxic ammonium in the blood. "@en . "Ureidoglutaric acid"@en . . . . . . "Median values for the terminal half-life was 5.6 hours (range 4.3-9.5)."@en . "L-N-Carbamoylglutamic acid"@en . . "30% bioavailability; Cmax, mean, 100 mg/kg dose = 2.6 \u03BCg/mL (range of 1.9 - 4.8) Carglumic acid is not subject to to intracellular degradation. "@en . . . "Following administration of a single radiolabeled oral dose of 100 mg/kg of body weight, 9% of the dose was excreted unchanged in the urine and up to 60% of the dose was excreted unchanged in the feces."@en . . "Acide carglumique"@en . . .