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3D schematic representation of E1

Ribbon diagram (Richardson diagram) of alpha-ketoglutarate dehydrogenase, also known as 2-oxoglutarate dehydrogenase, the E1 component of the alpha-ketoglutarate dehydrogenase complex, also known as 2-oxoglutarate dehydrogenase complex. E1 is a mitochondrial enzyme encoded by the OGDH gene. Image by Jawahar Swaminathan and MSD staff at the European Bioinformatics Institute. (Courtesy of Wikimedia Commons. Public domain.)

Associated Disorders

  • DOOR syndrome
  • Dihydrolipoyl dehydrogenase deficiency
  • Eronen syndrome
  • Ketoacidotic crisis
  • Lactic acidosis
  • Maple syrup urine disease