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Key Documents

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C4375

Sigma-Aldrich

N-Carbamyl-L-glutamic acid

Sinónimos:

N-(aminocarbonyl)-L-glutamic acid

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About This Item

Fórmula empírica (notación de Hill):
C6H10N2O5
Número de CAS:
1188-38-1
Peso molecular:
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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Categorías relacionadas

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_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

dust mask type N95 (US), Eyeshields, Gloves

Certificados de análisis (COA)

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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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