Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency

  title={Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency},
  author={Sven Sauer},
  journal={Journal of Inherited Metabolic Disease},
  • 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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WikiJournal of Science
Lysine plays several roles in humans, most importantly proteinogenesis, but also in the crosslinking of collagen polypeptides, uptake of essential mineral nutrients, and in the production of carnitine, which is key in fatty acid metabolism.


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