Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency

@article{Sauer2007BiochemistryAB,
  title={Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency},
  author={Sven Sauer},
  journal={Journal of Inherited Metabolic Disease},
  year={2007},
  volume={30},
  pages={673-680}
}
  • S. Sauer
  • Published 21 September 2007
  • Biology
  • Journal of Inherited Metabolic Disease
SummaryGlutaryl-CoA dehydrogenase (GCDH) is a central enzyme in the catabolic pathway of l-tryptophan, l-lysine, and l-hydroxylysine which catalyses the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO2. Glutaryl-CoA dehydrogenase deficiency (GDD) is an autosomal recessive disease characterized by the accumulation of glutaric and 3-hydroxyglutaric acids in tissues and body fluids. Untreated patients commonly present with severe striatal degeneration during encephalopathic crises… 
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