Increased glutamate receptor and transporter expression in the cerebral cortex and striatum of gcdh-/- mice: possible implications for the neuropathology of glutaric acidemia type... von Lagranha, Valeska Lizzi, Matte, Ursula, de Carvalho, Talita Giacomet, Seminotti, Bianca, Pereira, Carolina Coffi, Koeller, David M, Woontner, Michael, Goodman, Stephen I, de Souza, Diogo Onofre Gomes, Wajner, Moacir

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Glutaric acidemia type II: gene structure and mutations of the electron transfer flavoprotein:ubiquinone oxidoreductase (ETF:QO) gene von Goodman, Stephen I, Binard, Robert J, Woontner, Michael R, Frerman, Frank E

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An explanation for metabolite excretion in high- and low-excretor patients with glutaric acidemia type 1 von Goodman, Stephen I., Woontner, Michael

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In Memoriam von Van Hove, Johan L. K., Thomas, Janet A., McCandless, Shawn E., Woontner, Michael, Coughlin, Curtis

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In Memoriam von Van Hove, Johan L. K., Thomas, Janet A., McCandless, Shawn E., Woontner, Michael, Coughlin, Curtis

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In Memoriam von Johan L K Van Hove, Thomas, Janet A, McCandless, Shawn E, Woontner, Michael, Coughlin, Curtis

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In Memoriam Stephen Irwin Goodman, MD von Van Hove, Johan L. K., Thomas, Janet A., McCandless, Shawn E., Woontner, Michael, Coughlin, Curtis

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Increased susceptibility to quinolinic acid‐induced seizures and long‐term changes in brain oscillations in an animal model of glutaric acidemia type I von Barbieri Caus, Letícia, Pasquetti, Mayara Vendramin, Seminotti, Bianca, Woontner, Michael, Wajner, Moacir, Calcagnotto, Maria Elisa

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Long Lasting High Lysine Diet Aggravates White Matter Injury in Glutaryl-CoA Dehydrogenase Deficient (Gcdh−/−) Mice von Olivera-Bravo, Silvia, Seminotti, Bianca, Isasi, Eugenia, Ribeiro, César A., Leipnitz, Guilhian, Woontner, Michael, Goodman, Stephen I., Souza, Diogo, Barbeito, Luis, Wajner, Moacir

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Impairment of GABAergic system contributes to epileptogenesis in glutaric acidemia type I von Vendramin Pasquetti, Mayara, Meier, Letícia, Loureiro, Samanta, Ganzella, Marcelo, Junges, Bernardo, Barbieri Caus, Letícia, Umpierrez Amaral, Alexandre, Koeller, David M., Goodman, Stephen, Woontner, Michael, Gomes de Souza, Diogo Onofre, Wajner, Moacir, Calcagnotto, Maria Elisa

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In Memoriam von Van Hove, Johan L K, Thomas, Janet A, McCandless, Shawn E, Woontner, Michael, Coughlin, Curtis

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Experimental evidence that bioenergetics disruption is not mainly involved in the brain injury of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload von Amaral, Alexandre Umpierrez, Cecatto, Cristiane, Seminotti, Bianca, Ribeiro, César Augusto, Lagranha, Valeska Lizzi, Pereira, Carolina Coffi, de Oliveira, Francine Hehn, de Souza, Diogo Gomes, Goodman, Stephen, Woontner, Michael, Wajner, Moacir

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REVIEW: Practical strategies to maintain anabolism by intravenous nutritional management in children with inborn metabolic diseases von Kripps, Kimberly A., Baker, Peter R., Thomas, Janet A., Skillman, Heather E., Bernstein, Laurie, Gaughan, Sommer, Burns, Casey, Coughlin, Curtis R., McCandless, Shawn E., Larson, Austin A., Kochar, Aaina, Stillman, Chelsey F., Wymore, Erica M., Hendricks, Ellie G., Woontner, Michael, Van Hove, Johan L.K.

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Striatal neuronal death mediated by astrocytes from the Gcdh-/- mouse model of glutaric acidemia type I von Olivera-Bravo, Silvia, Ribeiro, César A J, Isasi, Eugenia, Trías, Emiliano, Leipnitz, Guilhian, Díaz-Amarilla, Pablo, Woontner, Michael, Beck, Cheryl, Goodman, Stephen I, Souza, Diogo, Wajner, Moacir, Barbeito, Luis

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Experimental evidence that overexpression of NR2B glutamate receptor subunit is associated with brain vacuolation in adult glutaryl-CoA dehydrogenase deficient mice: A potential ro... von Rodrigues, Marília Danyelle Nunes, Seminotti, Bianca, Amaral, Alexandre Umpierrez, Leipnitz, Guilhian, Goodman, Stephen Irwin, Woontner, Michael, de Souza, Diogo Onofre Gomes, Wajner, Moacir

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Impairment of GABA ergic system contributes to epileptogenesis in glutaric acidemia type I von Vendramin Pasquetti, Mayara, Meier, Letícia, Loureiro, Samanta, Ganzella, Marcelo, Junges, Bernardo, Barbieri Caus, Letícia, Umpierrez Amaral, Alexandre, Koeller, David M., Goodman, Stephen, Woontner, Michael, Gomes de Souza, Diogo Onofre, Wajner, Moacir, Calcagnotto, Maria Elisa

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A diet-induced mouse model for glutaric aciduria type I von Zinnanti, William J., Lazovic, Jelena, Wolpert, Ellen B., Antonetti, David A., Smith, Michael B., Connor, James R., Woontner, Michael, Goodman, Stephen I., Cheng, Keith C.

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Higher Vulnerability of Menadione-Exposed Cortical Astrocytes of Glutaryl-CoA Dehydrogenase Deficient Mice to Oxidative Stress, Mitochondrial Dysfunction, and Cell Death: Implicati... von Rodrigues, Marília Danyelle Nunes, Seminotti, Bianca, Zanatta, Ângela, de Mello Gonçalves, Aline, Bellaver, Bruna, Amaral, Alexandre Umpierrez, Quincozes-Santos, André, Goodman, Stephen Irwin, Woontner, Michael, Souza, Diogo Onofre, Wajner, Moacir

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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... von Seminotti, Bianca, Ribeiro, Rafael Teixeira, Amaral, Alexandre Umpierrez, da Rosa, Mateus Struecker, Pereira, Carolina Coffi, Leipnitz, Guilhian, Koeller, David M, Goodman, Stephen, Woontner, Michael, Wajner, Moacir

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Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I von Zinnanti, William J, Lazovic, Jelena, Housman, Cathy, LaNoue, Kathryn, O'Callaghan, James P, Simpson, Ian, Woontner, Michael, Goodman, Stephen I, Connor, James R, Jacobs, Russell E, Cheng, Keith C

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