General Information of This Metabolic Reaction (MR) (ID: MR005601)
Formula
Reactant Beta-Ketophenylbutyryl-CoA Product Phenylacetyl-CoA
Reactant Info Product Info
Metabolic Enzyme Medium-chain specific acyl-CoA dehydrogenase, mitochondrial (ACADM) DME Info
Other MR(s) Related to The Reactant of This MR
Other MR(s) That Produce The Reactant of This MR
MR ID Reactant Product MR Type Related Drug REF
MR005599 Beta-Hydroxyphenylbutyryl-CoA Beta-Ketophenylbutyryl-CoA Unclear Sodium phenylbutyrate [1]
MR013769 Beta-Hydroxyphenylbutyryl-CoA Beta-Ketophenylbutyryl-CoA Unclear - Unclear phenylbutyrate [2]
Other MR(s) That Metabolize The Reactant of This MR
MR ID Reactant Product MR Type Related Drug REF
MR005600 Beta-Ketophenylbutyryl-CoA Acetyl-CoA Unclear Sodium phenylbutyrate [1]
MR013771 Beta-Ketophenylbutyryl-CoA Acetyl-CoA Unclear - Unclear phenylbutyrate [2]
Other MR(s) Related to The Product of This MR
Other MR(s) That Produce The Product of This MR
MR ID Reactant Product MR Type Related Drug REF
MR005606 Sodium phenylbutyrate Phenylacetyl-CoA Oxidation - Oxidationn Sodium phenylbutyrate [1]
MR005605 PKB-CoA PA-CoA Unclear Sodium phenylbutyrate [3]
Other MR(s) That Metabolize The Produtc of This MR
MR ID Reactant Product MR Type Related Drug REF
MR005607 Phenylacetyl-CoA Phenylacetylglutamine Unclear Sodium phenylbutyrate [1]
References
1 Evidence for involvement of medium chain acyl-CoA dehydrogenase in the metabolism of phenylbutyrate
2 Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control
3 Identification of enzymes involved in oxidation of phenylbutyrate

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