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Wall-eyed stereoview of hETHE1

Wall-eyed stereoview of hETHE1 showing secondary structure elements and the mono iron-containing active site. Helices are blue, beta-strands are yellow, and the iron is an orange sphere. (Source: Pettinati I, Brem J, McDonough MA, Schofield CJ. Crystal structure of human persulfide dioxygenase: structural basis of ethylmalonic encephalopathy. Hum Mol Genet 2015;24[9]:2458-69. Creative Commons Attribution [CC BY 4.0] license, creativecommons.org/licenses/by/4.0)

Associated Disorders

  • fatty acid oxidation disorder
  • medium-chain acyl-CoA dehydrogenase deficiency
  • metabolic acidosis
  • orthostatic acrocyanosis