Chronic exposure to sulfide causes accelerated degradation of cytochrome c oxidase in ethylmalonic encephalopathy.

Abstract

Ethylmalonic encephalopathy (EE) is an autosomal recessive, invariably fatal disorder associated with mutations in ETHE1, a gene encoding a mitochondrial sulfur dioxygenase (SDO). The main consequence of the absence of Ethe1-SDO is the accumulation of sulfide (H(2)S) in critical tissues, including colonic mucosa, liver, muscle, and brain. To make progress… (More)
DOI: 10.1089/ars.2010.3520

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Cite this paper

@article{Meo2011ChronicET, title={Chronic exposure to sulfide causes accelerated degradation of cytochrome c oxidase in ethylmalonic encephalopathy.}, author={Ivano Di Meo and Gigliola Fagiolari and Alessandro Prelle and Carlo Viscomi and Massimo Zeviani and Valeria Tiranti}, journal={Antioxidants & redox signaling}, year={2011}, volume={15 2}, pages={353-62} }