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Merck
CN

C4375

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
UNSPSC Code:
12352209
eCl@ss:
32160406
PubChem Substance ID:
24892688
NACRES:
NA.26
MDL number:
MFCD00047874

Key Documents

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Product Name

N-Carbamyl-L-glutamic acid,

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

200

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

11 - Combustible Solids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

dust mask type N95 (US), Eyeshields, Gloves

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Certificates of Analysis (COA)

Lot/Batch Number
0000326749
0000326749
SLCP9189
SLCF4524
SLCC9964

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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
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.
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
Ljubica Caldovic et al.
The Journal of pediatrics, 145(4), 552-554 (2004-10-14)
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 ammonia rose following ammonium tracer ingestion before treatment
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