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3D schematic representation of E3

Ribbon diagram (Richardson diagram) of dihydrolipoamide dehydrogenase (DLD), also known as dihydrolipoyl dehydrogenase, the E3 component of the alpha-ketoglutarate dehydrogenase complex (also known as 2-oxoglutarate dehydrogenase complex). E3 is a mitochondrial enzyme encoded by the DLD gene. Image by Source: (Ciszak et al 2006). 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