product name
N-氨基甲酰-L-谷氨酸,
检测方案
≥98.0% (TLC)
质量水平
形式
powder
颜色
white
应用
cell analysis
储存温度
2-8°C
SMILES字符串
NC(=O)N[C@@H](CCC(O)=O)C(O)=O
InChI
1S/C6H10N2O5/c7-6(13)8-3(5(11)12)1-2-4(9)10/h3H,1-2H2,(H,9,10)(H,11,12)(H3,7,8,13)/t3-/m0/s1
InChI key
LCQLHJZYVOQKHU-VKHMYHEASA-N
基因信息
human ... CPS1(1373)
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生化/生理作用
N-氨基甲酰-L-谷氨酸(氨甲酰谷氨酸)被用于研究其作为替代疗法对N-乙酰谷氨酸合成酶(NAGS)缺乏症的治疗潜力,并用于治疗高氨血症。
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
个人防护装备
dust mask type N95 (US), Eyeshields, Gloves
PloS one, 8(7), e60581-e60581 (2013-07-19)
Most conditions detected by expanded newborn screening result from deficiency of one of the enzymes that degrade acyl-coenzyme A (CoA) esters in mitochondria. The role of acyl-CoAs in the pathophysiology of these disorders is poorly understood, in part because CoA
Journal of inherited metabolic disease, 28(2), 241-244 (2005-05-07)
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 (CPS). This finally leads to hyperammonaemia.
Neonatology, 97(3), 286-290 (2009-11-06)
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. It requires a rapid and
Amino acids, 39(3), 831-839 (2010-03-10)
This study determined effects of dietary supplementation with L-arginine (Arg) or N-carbamylglutamate (NCG) on intestinal health and growth in early-weaned pigs. Eighty-four Landrace x Yorkshire pigs (average body weight of 5.56+/-0.07 kg; weaned at 21 days of age) were fed
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
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