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Wall-eyed stereoview of hETHE1 (blue) and A thaliana ETHE1-like enzyme

Wall-eyed stereoview of the superposition of hETHE1 (blue) and the A thaliana ETHE1-like enzyme (red) (RMSD 1.43 Å over 230 Cα atoms). The latter is from Arabidopsis thaliana, the thale cress (or mouse-ear cress or arabidopsis), a small plant from the mustard family (Brassicaceae) native to Eurasia and Africa and now used as a model organism for plant genetics. The structures reveal very similar overall folds except for small differences in the beta2–beta3 and beta9–beta10 loops. (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