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  • lipoic
  • Lipoic acid serves as co-factor to the acetoin dehydrogenase complex catalyzing the conversion of acetoin (3-hydroxy-2-butanone) to acetaldehyde and acetyl coenzyme A, in some bacteria, allowing acetoin to be used as the sole carbon source. (wikipedia.org)
  • Glutathionylation "protects" the lipoic acid of the E2 domain from undergoing oxidative damage, which helps spare the Oxoglutarate dehydrogenase complex from oxidative stress. (wikipedia.org)
  • deficiency
  • 53 Dihydrolipoamide dehydrogenase (DLD) deficiency is a very rare condition that can vary in age of onset, symptoms and severity. (malacards.org)
  • A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia. (wikipedia.org)
  • People with pyruvate dehydrogenase deficiency usually have neurological problems as well. (wikipedia.org)
  • Because of the severe health effects, many individuals with pyruvate dehydrogenase deficiency do not survive past childhood, although some may live into adolescence or adulthood. (wikipedia.org)
  • Pyruvate dehydrogenase deficiency is characterized by the buildup of a chemical called lactic acid in the body and a variety of neurological problems. (wikipedia.org)
  • Disorders of fatty acid oxidation Medium chain acylCoA dehydrogenase deficiency (MCAD) Familial Leucine sensitive hypoglycemia Accidental ingestions including pharmacy misfills Sulfonylureas, propranolol and others Ethanol (mouthwash, alcoholic beverages) By far, the most common cause of severe hypoglycemia in this age range is insulin injected for type 1 diabetes. (wikipedia.org)
  • Krebs
  • By controlling the amount of available reducing equivalents generated by the Krebs cycle, Oxoglutarate dehydrogenase has a downstream regulatory effect on oxidative phosphorylation and ATP production. (wikipedia.org)
  • Inhibition
  • In the presence of a high concentration of free radical species, Oxoglutarate dehydrogenase undergoes fully reversible free radical mediated inhibition. (wikipedia.org)
  • It is believed that the temporary inhibition of mitochondrial function stems from the reversible glutathionylation of the E2-lipoac acid domain of Oxoglutarate dehydrogenase. (wikipedia.org)
  • The temporary inhibition period sparks a stronger up-regulation response, allowing an increased level of oxoglutarate dehydrogenase activity to compensate for the acute stress exposure. (wikipedia.org)
  • activity
  • Oxoglutarate dehydrogenase activity is turned off in the presence of free radicals in order to protect the enzyme from damage. (wikipedia.org)
  • In addition to free radicals and the mitochondrial redox state, Oxoglutarate dehydrogenase activity is also regulated by ATP/ADP ratios, the ratio of Succinyl-CoA to CoA-SH, and the concentrations of various metal ion cofactors (Mg2+, Ca2+). (wikipedia.org)
  • citric acid
  • Acetyl-CoA may then be used in the citric acid cycle to carry out cellular respiration, so pyruvate dehydrogenase contributes to linking the glycolysis metabolic pathway to the citric acid cycle and releasing energy via NADH. (wikipedia.org)