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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. 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
Reduction of Na+, K+-ATPase activity and expression in cerebral cortex of glutaryl-CoA dehydrogenase deficient mice: a possible mechanism for brain injury in glutaric aciduria type I.
Amaral AU, Seminotti B, Cecatto C, Fernandes CG, Busanello EN, Zanatta Â, Kist LW, Bogo MR, de Souza DO, Woontner M, Goodman S, Koeller DM, Wajner M. Amaral AU, et al. Mol Genet Metab. 2012 Nov;107(3):375-82. doi: 10.1016/j.ymgme.2012.08.016. Epub 2012 Aug 29. Mol Genet Metab. 2012. PMID: 22999741
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. 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
Increased glutamate receptor and transporter expression in the cerebral cortex and striatum of gcdh-/- mice: possible implications for the neuropathology of glutaric acidemia type I.
Lagranha VL, Matte U, de Carvalho TG, Seminotti B, Pereira CC, Koeller DM, Woontner M, Goodman SI, de Souza DO, Wajner M. Lagranha VL, et al. PLoS One. 2014 Mar 4;9(3):e90477. doi: 10.1371/journal.pone.0090477. eCollection 2014. PLoS One. 2014. PMID: 24594605 Free PMC article.
Animal models for glutaryl-CoA dehydrogenase deficiency.
Koeller DM, Sauer S, Wajner M, de Mello CF, Goodman SI, Woontner M, Mühlhausen C, Okun JG, Kölker S. Koeller DM, et al. J Inherit Metab Dis. 2004;27(6):813-8. doi: 10.1023/B:BOLI.0000045763.52907.5e. J Inherit Metab Dis. 2004. PMID: 15505386
132 results