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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. Among authors: woontner m. 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. Among authors: woontner m. 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
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: woontner m. 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: woontner m. 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
Disturbance of the glutamatergic system by glutaric acid in striatum and cerebral cortex of glutaryl-CoA dehydrogenase-deficient knockout mice: possible implications for the neuropathology of glutaric acidemia type I.
Busanello EN, Fernandes CG, Martell RV, Lobato VG, Goodman S, Woontner M, de Souza DO, Wajner M. Busanello EN, et al. Among authors: woontner m. J Neurol Sci. 2014 Nov 15;346(1-2):260-7. doi: 10.1016/j.jns.2014.09.003. Epub 2014 Sep 16. J Neurol Sci. 2014. PMID: 25241940
Experimental evidence that bioenergetics disruption is not mainly involved in the brain injury of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload.
Amaral AU, Cecatto C, Seminotti B, Ribeiro CA, Lagranha VL, Pereira CC, de Oliveira FH, de Souza DG, Goodman S, Woontner M, Wajner M. Amaral AU, et al. Among authors: woontner m. Brain Res. 2015 Sep 16;1620:116-29. doi: 10.1016/j.brainres.2015.05.013. Epub 2015 May 18. Brain Res. 2015. PMID: 25998543
Experimental evidence that overexpression of NR2B glutamate receptor subunit is associated with brain vacuolation in adult glutaryl-CoA dehydrogenase deficient mice: A potential role for glutamatergic-induced excitotoxicity in GA I neuropathology.
Rodrigues MD, Seminotti B, Amaral AU, Leipnitz G, Goodman SI, Woontner M, de Souza DO, Wajner M. Rodrigues MD, et al. Among authors: woontner m. J Neurol Sci. 2015 Dec 15;359(1-2):133-40. doi: 10.1016/j.jns.2015.10.043. Epub 2015 Oct 28. J Neurol Sci. 2015. PMID: 26671102
38 results