N-氨基甲酰-L-谷氨酸
N-氨基甲酰-L-谷氨酸结构式
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常用名 | N-氨基甲酰-L-谷氨酸 | 英文名 | Carglumic Acid |
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| CAS号 | 1188-38-1 | 分子量 | 190.15400 | |
| 密度 | 1.499g/cm3 | 沸点 | 438.1ºC at 760 mmHg | |
| 分子式 | C6H10N2O5 | 熔点 | 174° | |
| MSDS | 中文版 美版 | 闪点 | 218.8ºC | |
| 符号 |
GHS07 |
信号词 | Warning |
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Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia.
Eur. J. Pediatr. 169(1) , 133-4, (2010)
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Pricing of orphan drugs.
Lancet 373(9662) , 462, (2009)
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Effect of dietary arginine and N-carbamoylglutamate supplementation on reproduction and gene expression of eNOS, VEGFA and PlGF1 in placenta in late pregnancy of sows.
Anim. Reprod. Sci. 132(3-4) , 187-92, (2012) 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... |
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Oral N-carbamylglutamate supplementation increases protein synthesis in skeletal muscle of piglets.
J. Nutr. 137(2) , 315-9, (2007) This study investigated the potential mechanisms by which oral supplementation of N-carbamylglutamate (NCG), an analogue of endogenous N-acetylglutamate (an activator of arginine synthesis) increases growth rate in sow-reared piglets. Two piglets of equal bod... |
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N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers.
Pediatr. Res. 64(2) , 213-7, (2008) N-acetylglutamate (NAG) is an endogenous essential cofactor for conversion of ammonia to urea in the liver. Deficiency of NAG causes hyperammonemia and occurs because of inherited deficiency of its producing enzyme, NAG synthase (NAGS), or interference with i... |
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Effects of dietary L-arginine or N-carbamylglutamate supplementation during late gestation of sows on the miR-15b/16, miR-221/222, VEGFA and eNOS expression in umbilical vein.
Amino Acids 42(6) , 2111-9, (2012) Placental vascular formation and blood flow are crucial for fetal survival, growth and development, and arginine regulates vascular development and function. This study determined the effects of dietary arginine or N-carbamylglutamate (NCG) supplementation du... |
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Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate.
J. Pediatr. 145(4) , 552-4, (2004) 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 a... |
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N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria.
Neonatology 97(3) , 286-90, (2010) In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems. Hyperammonemia is a true neonatal emergency with high mortality and neurological complications in most survivors. ... |
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N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia.
J. Inherit. Metab. Dis. 28(2) , 241-4, (2005) In patients with propionic aciduria, the accumulating metabolite propionyl-CoA causes a disturbance of the urea cycle via the inhibition of N-acetylglutamate synthesis. Lack of this allosteric activator results in an inhibition of carbamoylphosphate synthase ... |
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N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria.
Mol. Genet. Metab. 79(4) , 303-4, (2003) 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 ... |