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C4375

Sigma-Aldrich

N-Carbamyl-L-glutamic acid

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

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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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
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
Sema Kalkan Ucar et al.
Metabolic brain disease, 24(3), 409-414 (2009-08-19)
Maple syrup urine disease (MSUD) is a defect in the catabolism of the branched-chain amino acids; leucine, isoleucine, and valine. Affected patients may also develop hyperammonaemia of unknown etiology. This report describes a four-year-old girl with MSUD, who presented with
Nathalie Guffon et al.
The Journal of pediatrics, 147(2), 260-262 (2005-08-30)
In a prospective study, patients with a suspected urea cycle defect underwent oral N-carbamoyl-L-glutamic acid loading testing. In patients with subsequently confirmed N-acetylglutamate synthase deficiency, hyperammonemia normalized within 8 hours. This test may be useful in the early diagnosis of
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