About: Carglumic Acid     Goto   Sponge   NotDistinct   Permalink

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AttributesValues
rdf:type
rdfs:label
  • Carglumic Acid
rdfs:subClassOf
Concept_In_Subset
Semantic_Type
  • Pharmacologic Substance
Preferred_Name
  • Carglumic Acid
PubMedID_Primary_Reference
  • 18516804
UMLS_CUI
  • C1318649
CAS_Registry
  • 1188-38-1
Accepted_Therapeutic_Use_For
  • N-acetylglutamate synthase (NAGS) Deficiency Hyperammonemia
FDA_UNII_Code
  • 5L0HB4V1EW
Contributing_Source
  • FDA
Chemical_Formula
  • C6H10N2O5
Legacy_Concept_Name
  • Carglumic_Acid
FULL_SYN
  • CARGLUMIC ACIDPTFDA5L0HB4V1EW
  • Carglumic AcidPTNCI
  • OE 312CNNCI
  • N-CarbamylglutamateSYNCI
  • CarbagluBRNCI
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.NCI
code
  • C76884
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