Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency

@article{Sauer2007BiochemistryAB,
  title={Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency},
  author={S. Sauer},
  journal={Journal of Inherited Metabolic Disease},
  year={2007},
  volume={30},
  pages={673-680}
}
  • S. Sauer
  • Published 2007
  • Biology, Medicine
  • 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… Expand
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12 Citations
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12 Citations

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Citation Type

Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I
Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice.
Neurotoxic Effects of trans-Glutaconic Acid in Rats
Disruption of mitochondrial homeostasis in organic acidurias: insights from human and animal studies
Astrocytic proliferation and mitochondrial dysfunction induced by accumulated glutaric acidemia I (GAI) metabolites: Possible implications for GAI pathogenesis
Lysine: Biosynthesis, catabolism and roles
Metabolic shift underlies recovery in reversible infantile respiratory chain deficiency
Metabolic shift underlies recovery in reversible infantile respiratory chain deficiency
Glutaric aciduria type 1 in South Africa-high incidence of glutaryl-CoA dehydrogenase deficiency in black South Africans.
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