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C4375

Sigma-Aldrich

N-Carbamyl-L-glutamic acid

≥98.0% (TLC)

Synonym(s):

N-(aminocarbonyl)-L-glutamic acid

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About This Item

Empirical Formula (Hill Notation):
C6H10N2O5
CAS Number:
1188-38-1
Molecular Weight:
190.15
MDL number:
MFCD00047874
UNSPSC Code:
12352209
eCl@ss:
32160406
PubChem Substance ID:
24892688
NACRES:
NA.26

product name

N-Carbamyl-L-glutamic acid,

Assay

≥98.0% (TLC)

Quality Level

200

form

powder

color

white

application(s)

cell analysis

storage temp.

2-8°C

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

Gene Information

human ... CPS1(1373)

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Biochem/physiol Actions

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.

Storage Class Code

11 - Combustible Solids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Personal Protective Equipment

dust mask type N95 (US), Eyeshields, Gloves

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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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.
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
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
X Wu et al.
Animal reproduction science, 132(3-4), 187-192 (2012-06-12)
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
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