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Target intelligence / Profile preview
Glutaryl-CoA dehydrogenase (GCDH)
- Target
- GCDH
- Molecular classification
- Enzyme, Oxidoreductase
01
Overview
Glutaryl-CoA dehydrogenase (GCDH) is a mitochondrial enzyme that catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide. This reaction is a key step in the catabolic pathway for the amino acids L-lysine, L-hydroxylysine, and L-tryptophan. Deficiency or dysfunction due to mutations in GCDH causes glutaric aciduria type I (GA-I), an autosomal recessive metabolic disorder characterized by accumulation of glutaric acid and related metabolites.
02
Mechanism of action
N/A
03
Biological functions
Amino acid metabolismLysine degradationTryptophan degradation
04
Disease associations
Glutaric aciduria type INeurometabolic disorders
05
Safety considerations
Neurotoxicity due to metabolite accumulationEncephalitis-like crises
06
Biomarkers
Glutaric acidRelated metabolites
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