API reference
Carglumic acid
- CAS number
- 1188-38-1
- Molecular formula
- C6H10N2O5
- Molecular weight
- 190.15 g/mol

About this molecule
Carglumic Acid is an orally active, synthetic structural analogue of N-acetylglutamate (NAG) and carbamoyl phosphate synthetase 1 (CPS 1) activator, with ammonia lowering activity. NAG, which is formed by the hepatic enzyme N-acetylglutamate synthase (NAGS), is an essential allosteric activator of the enzyme carbamoyl phosphate synthetase 1 (CPS 1). CPS 1 plays an essential role in the urea cycle and converts ammonia into urea. Upon oral administration, carglumic acid can replace NAG in NAGS deficient patients and activates CPS 1, which prevents hyperammonaemia.
Source: NCI Thesaurus (NCIt), via PubChem.
Identifiers
- IUPAC name
- (2S)-2-(carbamoylamino)pentanedioic acid
- InChIKey
- LCQLHJZYVOQKHU-VKHMYHEASA-N
- SMILES
- C(CC(=O)O)[C@@H](C(=O)O)NC(=O)N
- PubChem CID
- 121396
Also known as
Carglumic acid · N-Carbamyl-L-glutamic acid · Carbaglu · N-Carbamoyl-L-glutamic acid · (2S)-2-(carbamoylamino)pentanedioic acid · Ureidoglutaric acid · Carbamylglutamic acid · Carbamino-L-glutamic acid
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