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In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this disorder.
Fernandes CG, da Rosa MS, Seminotti B, Pierozan P, Martell RW, Lagranha VL, Busanello EN, Leipnitz G, Wajner M. Fernandes CG, et al. Among authors: da rosa ms. Mol Genet Metab. 2013 Jun;109(2):144-53. doi: 10.1016/j.ymgme.2013.03.017. Epub 2013 Apr 6. Mol Genet Metab. 2013. PMID: 23611578
Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation.
Seminotti B, Amaral AU, da Rosa MS, Fernandes CG, Leipnitz G, Olivera-Bravo S, Barbeito L, Ribeiro CA, de Souza DO, Woontner M, Goodman SI, Koeller DM, Wajner M. Seminotti B, et al. Among authors: da rosa ms. Mol Genet Metab. 2013 Jan;108(1):30-9. doi: 10.1016/j.ymgme.2012.11.001. Epub 2012 Nov 9. Mol Genet Metab. 2013. PMID: 23218171
Acute lysine overload provokes protein oxidative damage and reduction of antioxidant defenses in the brain of infant glutaryl-CoA dehydrogenase deficient mice: a role for oxidative stress in GA I neuropathology.
Seminotti B, Ribeiro RT, Amaral AU, da Rosa MS, Pereira CC, Leipnitz G, Koeller DM, Goodman S, Woontner M, Wajner M. Seminotti B, et al. Among authors: da rosa ms. J Neurol Sci. 2014 Sep 15;344(1-2):105-13. doi: 10.1016/j.jns.2014.06.034. Epub 2014 Jun 22. J Neurol Sci. 2014. PMID: 24996493
In vivo intracerebral administration of L-2-hydroxyglutaric acid provokes oxidative stress and histopathological alterations in striatum and cerebellum of adolescent rats.
da Rosa MS, João Ribeiro CA, Seminotti B, Teixeira Ribeiro R, Amaral AU, Coelho Dde M, de Oliveira FH, Leipnitz G, Wajner M. da Rosa MS, et al. Free Radic Biol Med. 2015 Jun;83:201-13. doi: 10.1016/j.freeradbiomed.2015.02.008. Epub 2015 Feb 17. Free Radic Biol Med. 2015. PMID: 25701435
3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency.
da Rosa MS, Seminotti B, Ribeiro CA, Parmeggiani B, Grings M, Wajner M, Leipnitz G. da Rosa MS, et al. Free Radic Res. 2016 Sep;50(9):997-1010. doi: 10.1080/10715762.2016.1214952. Epub 2016 Aug 23. Free Radic Res. 2016. PMID: 27430492
16 results