Urea Cycle Disorder Agents

Urea Cycle Disorder Agents
Accession Number

Not Available

DrugDrug Description
Phenylbutyric acidAn agent indicated for the adjunctive therapy for the management of chronic urea cycle disorders due to deficiencies in specific enzymes, including the neonatal-onset deficiency and late-onset disease with a history of hyperammonemic encephalopathy.
Glycerol phenylbutyrateA nitrogen-binding agent used to manage patients with urea cycle disorders who cannot be sufficiently managed amino acid supplementation and/or dietary restrictions.
Benzoic acidAn antimicrobial food additive.
Phenylacetic acidAn agent used as an adjunct to treat acute hyperammonemia and associated encephalopathy in adult and pediatric patients with deficiencies in enzymes of the urea cycle.
Drugs & Drug Targets
Phenylbutyric acidAromatic-amino-acid aminotransferasetarget
Phenylbutyric acidHistone deacetylasetarget
Glycerol phenylbutyratePancreatic triacylglycerol lipaseenzyme
Glycerol phenylbutyrateCytochrome P450 3A4enzyme
Glycerol phenylbutyrateCytochrome P450 2D6enzyme
Benzoic acidCocaine esterasetarget
Benzoic acidD-amino-acid oxidasetarget
Benzoic acidChlorocatechol 1,2-dioxygenasetarget
Benzoic acid2-hydroxy-6-oxo-7-methylocta-2,4-dienoate hydrolasetarget
Benzoic acidNon-heme chloroperoxidasetarget
Benzoic acidOxygen-insensitive NAD(P)H nitroreductasetarget
Benzoic acidHydroxyquinol 1,2-dioxygenasetarget
Benzoic acidReplication proteintarget
Benzoic acidSolute carrier organic anion transporter family member 2B1transporter
Benzoic acidSolute carrier family 22 member 8transporter
Benzoic acidMonocarboxylate transporter 1transporter
Benzoic acidSolute carrier family 15 member 1target
Phenylacetic acidLiver carboxylesterase 1enzyme