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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. 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
Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice.
Amaral AU, Cecatto C, Seminotti B, Zanatta Â, Fernandes CG, Busanello EN, Braga LM, Ribeiro CA, de Souza DO, Woontner M, Koeller DM, Goodman S, Wajner M. Amaral AU, et al. Among authors: fernandes cg. Mol Genet Metab. 2012 Sep;107(1-2):81-6. doi: 10.1016/j.ymgme.2012.04.015. Epub 2012 Apr 24. Mol Genet Metab. 2012. PMID: 22578804
67 results