CHEBI:71028 - carglumic acid

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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 0
Average Mass 190.15400
Monoisotopic Mass 190.059
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
An effector 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.
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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)
IUPAC Name
N-carbamoyl-L-glutamic acid
INNs Sources
acide carglumique WHO MedNet
ácido carglúmico WHO MedNet
acidum carglumicum WHO MedNet
carglumic acid KEGG DRUG
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
Database Links Databases
3068 DrugCentral
C05829 KEGG COMPOUND
Carglumic_acid Wikipedia
D07130 KEGG DRUG
DB06775 DrugBank
HMDB0015673 HMDB
N-CARBAMYL-L-GLUTAMATE MetaCyc
View more database links
Registry Numbers Types Sources
1188-38-1 CAS Registry Number KEGG DRUG
1188-38-1 CAS Registry Number ChemIDplus
1727929 Reaxys Registry Number Reaxys
Citations Waiting for Citations Types Sources
15055204 PubMed citation Europe PMC
17421020 PubMed citation Europe PMC
18234091 PubMed citation Europe PMC
18516804 PubMed citation Europe PMC
18604903 PubMed citation Europe PMC
20410539 PubMed citation Europe PMC
21207059 PubMed citation Europe PMC
21225012 PubMed citation Europe PMC
21403788 PubMed citation Europe PMC
21941437 PubMed citation Europe PMC
22858088 PubMed citation Europe PMC
Last Modified
22 February 2017