Carglumic Acid
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MedKoo CAT#: 317372

CAS#: 1188-38-1

Description: Carglumic acid is an orphan drug, marketed by Orphan Europe under the trade name Carbaglu. Carglumic acid is used for the treatment of hyperammonaemia in patients with N-acetylglutamate synthase deficiency. The initial daily dose ranges from 100 to 250 mg/kg, adjusted thereafter to maintain normal plasma levels of ammonia.


Chemical Structure

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Carglumic Acid
CAS# 1188-38-1

Theoretical Analysis

MedKoo Cat#: 317372
Name: Carglumic Acid
CAS#: 1188-38-1
Chemical Formula: C6H10N2O5
Exact Mass: 190.05897
Molecular Weight: 190.154
Elemental Analysis: C, 37.90; H, 5.30; N, 14.73; O, 42.07

Price and Availability

Size Price Availability Quantity
10.0mg USD 80.0 2 Weeks
25.0mg USD 90.0 2 Weeks
50.0mg USD 120.0 2 Weeks
100.0mg USD 150.0 2 Weeks
200.0mg USD 250.0 2 Weeks
500.0mg USD 450.0 2 Weeks
1.0g USD 750.0 2 Weeks
2.0g USD 1250.0 2 Weeks
5.0g USD 2650.0 2 Weeks
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Synonym: OE312, OE-312, OE 312, AJ 266447, AJ-26647, AJ26647; Carglumic Acid.

IUPAC/Chemical Name: (2S)-2-(carbamoylamino)pentanedioic acid

InChi Key: LCQLHJZYVOQKHU-VKHMYHEASA-N

InChi Code: 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

SMILES Code: O=C(O)[C@@H](NC(N)=O)CCC(O)=O

Appearance: Solid powder

Purity: >98% (or refer to the Certificate of Analysis)

Shipping Condition: Shipped under ambient temperature as non-hazardous chemical. This product is stable enough for a few weeks during ordinary shipping and time spent in Customs.

Storage Condition: Dry, dark and at 0 - 4 C for short term (days to weeks) or -20 C for long term (months to years).

Solubility: Soluble in DMSO, not in water

Shelf Life: >2 years if stored properly

Drug Formulation: This drug may be formulated in DMSO

Stock Solution Storage: 0 - 4 C for short term (days to weeks), or -20 C for long term (months).

HS Tariff Code: 2934.99.9001

Preparing Stock Solutions

The following data is based on the product molecular weight 190.154 Batch specific molecular weights may vary from batch to batch due to the degree of hydration, which will affect the solvent volumes required to prepare stock solutions.

Recalculate based on batch purity %
Concentration / Solvent Volume / Mass 1 mg 5 mg 10 mg
1 mM 1.15 mL 5.76 mL 11.51 mL
5 mM 0.23 mL 1.15 mL 2.3 mL
10 mM 0.12 mL 0.58 mL 1.15 mL
50 mM 0.02 mL 0.12 mL 0.23 mL

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1: Matoori S, Leroux JC. Recent advances in the treatment of hyperammonemia. Adv
Drug Deliv Rev. 2015 Aug 1;90:55-68. doi: 10.1016/j.addr.2015.04.009. Epub 2015
Apr 17. Review. PubMed PMID: 25895618.

2: van Karnebeek C, Häberle J. Carbonic Anhydrase VA Deficiency. 2015 Apr 2. In:
Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Fong
CT, Mefford HC, Smith RJH, Stephens K, editors. GeneReviews® [Internet]. Seattle
(WA): University of Washington, Seattle; 1993-2015. Available from
http://www.ncbi.nlm.nih.gov/books/NBK284774/
PubMed PMID: 25834911.