Target type: molecularfunction
Catalysis of the reaction: glutaryl-CoA + 2 H+ + oxidized [electron-transfer flavoprotein] = (2E)-butenoyl-CoA + CO2 + reduced [electron-transfer flavoprotein]. [RHEA:13389]
Glutaryl-CoA dehydrogenase (GCDH) is an enzyme that catalyzes the fourth step in the catabolism of the amino acids lysine, hydroxylysine, and tryptophan. This step involves the oxidation of glutaryl-CoA to crotonyl-CoA, producing CO2 and reducing FAD to FADH2. The reaction occurs in the mitochondrial matrix and requires FAD as a cofactor. GCDH plays a crucial role in energy production by participating in the degradation of these amino acids, converting them into usable energy molecules. Mutations in the GCDH gene can lead to glutaric aciduria type 1 (GA1), a rare but serious metabolic disorder characterized by the accumulation of glutaric acid in body fluids. GA1 can cause neurological damage and developmental delay if left untreated.'
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| Protein | Definition | Taxonomy |
|---|---|---|
| Glutaryl-CoA dehydrogenase, mitochondrial | A glutaryl-CoA dehydrogenase, mitochondrial that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q92947] | Homo sapiens (human) |
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| balsalazide | balsalazide : A monohydroxybenzoic acid consisting of 5-aminosalicylic acid (mesalazine) linked to 4-aminobenzoyl-beta-alanine via an azo bond. balsalazide: a mesalamine 5-aminosalicylate prodrug; 99% of ingested drug remains intact through the stomach and is delivered to and activated in the colon; used for inflammatory bowel disease, ulcerative colitis and radiation-induced proctosigmoiditis but avoided in patients with known hypersensitivity reaction to salicylates or mesalamine; structure in first source |