API reference

Carglumic acid

CAS number
1188-38-1
Molecular formula
C6H10N2O5
Molecular weight
190.15 g/mol
Carglumic acid — 2D structure
2D structure (PubChem)

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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