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Beta-oxidation of the mitochondrial matrix

The altered bonds in each reaction are shown in red. Medium-chain acyl-CoA dehydrogenase deficiency works at the acyl-CoA dehydrogenase step with maximum activity for octanoyl-CoA as substrate. (Contributed by Dr. Jerry Vockley.)

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Associated Disorders

  • Hypoketotic hypoglycemia
  • Reye syndrome
  • Secondary carnitine deficiency
  • Sudden infant death syndrome