N-Carbamyl-L-Glutamic acid - CAS 1188-38-1
Catalog number:
1188-38-1
Category:
Inhibitor
Not Intended for Therapeutic Use. For research use only.
Molecular Formula:
C6H10N2O5
Molecular Weight:
190.15
COA:
Inquire
Targets:
Others
Description:
N-Carbamyl-L-Glutamic acid is an orphan drug which is applicated for the treatment of hyperammonaemia in patients that has N-acetylglutamate synthase deficiency. In Jul 2014, Carglumic acid received Orphan Drug status for Organic acidaemias in USA. In Sep 2015, Carglumic acid registered for Hyperammonaemia in Canada .
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Purity:
98%
Appearance:
White to Off-White Solid
Synonyms:
Carbaglu; Carbamino-L-glutamic Acid; Carbamylglutamic Acid; Carglumic Acid; N-Carbamoyl-L-glutamic Acid; N-Carbamoyl-S-glutamic Acid; N-Carbamyl-L-glutamic Acid; OE 312; Ureidoglutaric Acid; N-(Aminocarbonyl)-L-glutamic Acid;
Solubility:
DMSO (Slightly), Methanol (Slightly)
Storage:
-20°C Freezer
MSDS:
Inquire
Application:
the treatment of acute hyperammonemia
Quality Standard:
In-house standard
Shelf Life:
2 month in rt, long time
Quantity:
Milligrams-Grams
Boiling Point:
438.1ºC at 760 mmHg
Melting Point:
150-154°C
Density:
1.499g/cm3
InChIKey:
LCQLHJZYVOQKHU-VKHMYHEASA-N
InChI:
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
Canonical SMILES:
C(CC(=O)O)C(C(=O)O)NC(=O)N
Current Developer:
Originator:Orphan Europe
1.[Determination of N-carbamyl-L-glutamic acid in feedstuff by high performance liquid chromatography coupled with electrospray ionization tandem mass spectrometry].
Tang S, Wang Y, Wen P, Xin Z. Se Pu. 2014 Feb;32(2):184-8.
A method was developed for the determination of N-carbamyl-L-glutamic acid (NCG) in feedstuff by high performance liquid chromatography coupled with electrospray ionization tandem mass spectrometry (HPLC-ESI-MS/MS). The feedstuff samples were extracted with methanol, cleaned-up by a ProElut mixed-mode of strong anion exchange inverse column (PXA). Then the samples were separated with HPLC and detected with MS/MS in multiple reaction monitoring (MRM) mode via positive electrospray ionization (ESI+). The fragment ions of m/z 148.0 and m/z 84.0 were used for qualitative analysis, and m/z 130.0 was selected for quantitative analysis. The limit of detection for NCG was 24 microg/kg (S/N > 3), and the limit of quantification was 80 microg/kg (S/N > 10). The standard calibration curve was linear over the range of 20-1,000 microg/L, and the correlation coefficient was 0. 999 9. The recoveries of NCG in feedstuff were 104.0%, 103.5%, 95.3%, at the three spiked levels of 80, 200, 500 mg/kg with the relative standard deviations (RSDs) of 7.
2.A liver-specific defect of Acyl-CoA degradation produces hyperammonemia, hypoglycemia and a distinct hepatic Acyl-CoA pattern.
Gauthier N1, Wu JW, Wang SP, Allard P, Mamer OA, Sweetman L, Moser AB, Kratz L, Alvarez F, Robitaille Y, Lépine F, Mitchell GA. PLoS One. 2013 Jul 5;8(7):e60581. doi: 10.1371/journal.pone.0060581. Print 2013.
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 esters are intracellular and samples are not generally available from human patients. We created a mouse model of one such condition, deficiency of 3-hydroxy-3-methylglutaryl-CoA lyase (HL), in liver (HLLKO mice). HL catalyses a reaction of ketone body synthesis and of leucine degradation. Chronic HL deficiency and acute crises each produced distinct abnormal liver acyl-CoA patterns, which would not be predictable from levels of urine organic acids and plasma acylcarnitines. In HLLKO hepatocytes, ketogenesis was undetectable. Carboxylation of [2-(14)C] pyruvate diminished following incubation of HLLKO hepatocytes with the leucine metabolite 2-ketoisocaproate (KIC). HLLKO mice also had suppression of the normal hyperglycemic response to a systemic pyruvate load, a measure of gluconeogenesis.
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CAS 1188-38-1 N-Carbamyl-L-Glutamic acid

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