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Crystal structure of human persulfide dioxygenase: structural basis of ethylmalonic encephalopathy
The ethylmalonic encephalopathy protein 1 (ETHE1) catalyses the oxygen-dependent oxidation of glutathione persulfide (GSSH) to give persulfite and glutathione. Mutations to the hETHE1 gene compromise sulfide metabolism leading to the genetic disease ethylmalonic encephalopathy. hETHE1 is a mono-iron...
Autores principales: | Pettinati, Ilaria, Brem, Jürgen, McDonough, Michael A., Schofield, Christopher J. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4383860/ https://www.ncbi.nlm.nih.gov/pubmed/25596185 http://dx.doi.org/10.1093/hmg/ddv007 |
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