National Cancer Institute Thesaurus

Last uploaded: February 23, 2024
Preferred Name

Carglumic Acid

Synonyms

CARGLUMIC ACID

N-Carbamylglutamate

Carglumic Acid

Carbaglu

OE 312

Definitions

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.

ID

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C76884

Accepted_Therapeutic_Use_For

N-acetylglutamate synthase (NAGS) Deficiency Hyperammonemia

CAS_Registry

1188-38-1

Chemical_Formula

C6H10N2O5

code

C76884

Concept_In_Subset

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C63923

Contributing_Source

FDA

DEFINITION

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.

FDA_UNII_Code

5L0HB4V1EW

FULL_SYN

CARGLUMIC ACID

N-Carbamylglutamate

Carglumic Acid

Carbaglu

OE 312

label

Carglumic Acid

Legacy Concept Name

Carglumic_Acid

Preferred_Name

Carglumic Acid

prefixIRI

Thesaurus:C76884

PubMedID_Primary_Reference

18516804

Semantic_Type

Pharmacologic Substance

UMLS_CUI

C1318649

subClassOf

http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C78275

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