WARNING: This product is for research use only, not for human or veterinary use.
MedKoo CAT#: 317372
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.
MedKoo Cat#: 317372
Name: Carglumic Acid
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
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
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.
|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|
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
PubMed PMID: 25834911.