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Bioenergetics dysfunction, mitochondrial permeability transition pore opening and lipid peroxidation induced by hydrogen sulfide as relevant pathomechanisms underlying the neurological dysfunction characteristic of ethylmalonic encephalopathy.
Cardoso GMF, Pletsch JT, Parmeggiani B, Grings M, Glanzel NM, Bobermin LD, Amaral AU, Wajner M, Leipnitz G. Cardoso GMF, et al. Among authors: pletsch jt. Biochim Biophys Acta Mol Basis Dis. 2017 Sep;1863(9):2192-2201. doi: 10.1016/j.bbadis.2017.06.007. Epub 2017 Jun 15. Biochim Biophys Acta Mol Basis Dis. 2017. PMID: 28624490 Free article.
Bezafibrate prevents mitochondrial dysfunction, antioxidant system disturbance, glial reactivity and neuronal damage induced by sulfite administration in striatum of rats: Implications for a possible therapeutic strategy for sulfite oxidase deficiency.
Grings M, Moura AP, Parmeggiani B, Pletsch JT, Cardoso GMF, August PM, Matté C, Wyse ATS, Wajner M, Leipnitz G. Grings M, et al. Among authors: pletsch jt. Biochim Biophys Acta Mol Basis Dis. 2017 Sep;1863(9):2135-2148. doi: 10.1016/j.bbadis.2017.05.019. Epub 2017 May 18. Biochim Biophys Acta Mol Basis Dis. 2017. PMID: 28529047 Free article.