C4375
N-氨基甲酰-L-谷氨酸
≥98.0% (TLC)
别名:
N-(aminocarbonyl)-L-glutamic acid
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关于此项目
经验公式（希尔记法）:
C6H10N2O5
化学文摘社编号:
分子量:
190.15
UNSPSC Code:
12352209
eCl@ss:
32160406
PubChem Substance ID:
NACRES:
NA.26
MDL number:
产品名称
N-氨基甲酰-L-谷氨酸,
SMILES string
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
assay
≥98.0% (TLC)
form
powder
color
white
application(s)
cell analysis
storage temp.
2-8°C
Quality Level
Gene Information
human ... CPS1(1373)
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Biochem/physiol Actions
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。
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
dust mask type N95 (US), Eyeshields, Gloves
Xin Wu et al.
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
Luca Filippi et al.
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
Nicolas Gauthier et al.
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
B Gebhardt et al.
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.
Nicholas Ah Mew et al.
Pediatrics, 126(1), e208-e214 (2010-06-23)
The objective of this study was to determine whether N-carbamylglutamate (NCG) reduces plasma levels of ammonia and glutamine and increases the rate of ureagenesis in patients with propionic acidemia (PA). Identical 4-hour studies were performed before and immediately after a
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