EC Number   |
General Information |
Reference |
|---|
    1.3.8.6 | evolution |
glutaryl-CoA dehydrogenase belongs to the acyl-CoA dehydrogenase enzyme family |
723842 |
| 1.3.8.6 | evolution |
glutaryl-CoA dehydrogenase belongs to the acyl-CoA dehydrogenase enzyme family, phylogenetic tree, overview |
-, 723842 |
| 1.3.8.6 | evolution |
the enzyme is a member of the acyl-CoA dehydrogenase (ACD) family of flavoproteins |
724526 |
| 1.3.8.6 | malfunction |
defects in glutaryl-CoA dehydrogenase are involved in glutaric acidemia type 1, an inherited metabolic disorder which can cause macrocephaly, muscular rigidity, spastic paralysis and other progressive movement disorders in humans |
723842 |
| 1.3.8.6 | malfunction |
glutaric aciduria type I (GA-I) is an autosomal recessive neurometabolic disease caused by mutations in the GCDH gene that encodes for glutaryl-CoA dehydrogenase (GCDH), a flavoprotein involved in the metabolism of tryptophan, lysine and hydroxylysin |
762752 |
| 1.3.8.6 | malfunction |
in glutaric aciduria type 1, glutaryl-CoA dehydrogenase deficiency is responsible for the accumulation of glutaric acid and striatal degeneration, GA1-induced striatal degeneration is partially caspase-dependent, mechanism, overview |
-, 726334 |
| 1.3.8.6 | malfunction |
injection of (3H)-labeled 3-hydroxyglutaric acid into 6 week-old Gcdh knockout mice, a model of glutaric aciduria type 1, reveal a low recovery in kidney, liver, or brain tissue that does not differ from control mice. Significant amounts of 3-hydroxyglutaric acid are found to be excreted via the intestinal tract. Exposure of knockout mice to a high protein diet leads to an encephalopathic crisis, vacuolization in the brain, and death after 4-5 days. Under these conditions, high amounts of injected 3H-3-hydroxyglutaric acid are found in kidneys of Gcdh knockout mice, whereas the radioactivity recovered in brain and blood is reduced. The data demonstrate that under conditions mimicking encephalopathic crises the blood-brain barrier appears to remain intact |
702409 |
| 1.3.8.6 | metabolism |
glutaryl-CoA dehydrogenase (GCDH), a flavoprotein, is involved in the metabolism of tryptophan, lysine and hydroxylysin |
762752 |
| 1.3.8.6 | metabolism |
glutaryl-coenzyme A dehydrogenases involved in amino acid degradation catalyze both the dehydrogenation and decarboxylation of glutaryl-CoA to crotonoyl-CoA and CO2 |
711251 |
| 1.3.8.6 | metabolism |
the enzyme catalyzes an intermediate step in the metabolic breakdown of lysine and tryptophan |
-, 723842 |
