carglumic acid (CHEBI:71028)

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ChEBI Name	carglumic acid

ChEBI ID	CHEBI:71028

Definition	A urea that is the N-carbamoyl derivative of L-glutamic acid. An orphan drug used to treat a deficiency in the enzyme N-acetylglutamate synthase, which leads to acute hyperammonaemia.

Stars	This entity has been manually annotated by the ChEBI Team.

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Formula

C6H10N2O5

Net Charge

Average Mass

190.15400

Monoisotopic Mass

190.05897

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

InChIKey

LCQLHJZYVOQKHU-VKHMYHEASA-N

SMILES

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

Roles Classification
Chemical Role(s):	Bronsted acid A molecular entity capable of donating a hydron to an acceptor (Bronsted base). (via oxoacid )

Biological Role(s):	carbamylphosphate synthetase I activator Any compound that binds and activates the enzyme carbamylphosphate synthetase I.

Application(s):	orphan drug Any drug that has been developed specifically for treatment of a rare medical condition, the condition itself being known as an orphan disease.

ChEBI Ontology
Outgoing	carglumic acid (CHEBI:71028) has role carbamylphosphate synthetase I activator (CHEBI:71033) carglumic acid (CHEBI:71028) has role orphan drug (CHEBI:71031) carglumic acid (CHEBI:71028) is a N-acyl-L-glutamic acid (CHEBI:21650) carglumic acid (CHEBI:71028) is a ureas (CHEBI:47857) carglumic acid (CHEBI:71028) is conjugate acid of N-carbamoyl-L-glutamate(2−) (CHEBI:229697)

Incoming	N-carbamoyl-L-glutamate(2−) (CHEBI:229697) is conjugate base of carglumic acid (CHEBI:71028)

IUPAC Name

N-carbamoyl-L-glutamic acid

Synonyms	Sources
(2S)-2-(carbamoylamino)pentanedioic acid	HMDB
Carbamino-L-glutamic acid	ChemIDplus
Carbamylglutamic acid	ChemIDplus
L-N-Carbamoylglutamic acid	ChemIDplus
Ureidoglutaric acid	ChemIDplus

Brand Name	Source
Carbaglu	KEGG DRUG

Last Modified

20 March 2024