Results 231 to 240 of about 17,667 (261)

Higher Vulnerability of Menadione-Exposed Cortical Astrocytes of Glutaryl-CoA Dehydrogenase Deficient Mice to Oxidative Stress, Mitochondrial Dysfunction, and Cell Death: Implications for the Neurodegeneration in Glutaric Aciduria Type I

Molecular Neurobiology, 2017
M. Rodrigues, B. Seminotti, Â. Zanatta, A. Gonçalves, Bruna Bellaver, A. U. Amaral, André Quincozes-Santos, S. Goodman, M. Woontner, D. Souza, M. Wajner   +10 more
semanticscholar   +1 more source

Synthesis of Substrate Analogs for Glutaryl-CoA Dehydrogenase: 3-Thia-Glutaryl-CoA and 4-Nitrobutyryl-CoA

Microchemical Journal, 1997
Colleen M. Byron, Leonard Edwards, Patricia L. Kultgen   +2 more
openaire   +2 more sources

Impairment of neuromotor development and cognition associated with histopathological and neurochemical abnormalities in the cerebral cortex and striatum of glutaryl-CoA dehydrogenase deficient mice

Neurochemistry International
E. T. Castro, R. Ribeiro, A. Carvalho, D. Machado, Ângela Beatris Zemniaçak, Rafael Palavro, Sâmela de Azevedo Cunha, Tailine Quevedo Tavares, Diogo Onofre Gomes de Souza, Carlos Alexandre Netto, G. Leipnitz, A. U. Amaral, M. Wajner   +12 more
semanticscholar   +1 more source

Glutaric aciduria: Inherited deficiency of glutaryl-CoA dehydrogenase activity

Biochemical Medicine, 1975
Stephen I. Goodman, Jerry G. Kohlhoff
openaire   +2 more sources

Induction of Neuroinflammatory Response and Histopathological Alterations Caused by Quinolinic Acid Administration in the Striatum of Glutaryl-CoA Dehydrogenase Deficient Mice

Neurotoxicity research, 2017
A. U. Amaral, B. Seminotti, Janaina da Silva, Francine Hehn de Oliveira, R. Ribeiro, C. Vargas, G. Leipnitz, A. Santamaría, D. Souza, M. Wajner   +9 more
semanticscholar   +1 more source

l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload.

Biochimica et Biophysica Acta - Molecular Basis of Disease, 2019
Gilian Guerreiro, A. U. Amaral, R. Ribeiro, J. Faverzani, A. C. Groehs, A. Sitta, M. Deon, M. Wajner, C. Vargas   +8 more
semanticscholar   +1 more source

Intracerebral accumulation of glutaric and 3‐hydroxyglutaric acids secondary to limited flux across the blood–brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl‐CoA dehydrogenase deficiency

Journal of Neurochemistry, 2006
S. Sauer, J. Okun, G. Fricker, Anne Mahringer, I. Müller, Linda R. Crnic, C. Mühlhausen, G. Hoffmann, F. Hörster, S. Goodman, C. Harding, D. Koeller, S. Kölker   +12 more
semanticscholar   +1 more source

Increased glutarate production by blocking the glutaryl-CoA dehydrogenation pathway and a catabolic pathway involving l-2-hydroxyglutarate

Nature Communications, 2018
Chao Gao, Ping Xu, Wen Zhang
exaly  

Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance.

Biochimica et Biophysica Acta - Proteins and Proteomics, 2019
J. V. Ribeiro, Tânia G. Lucas, P. Bross, C. Gomes, Bárbara J Henriques   +4 more
semanticscholar   +1 more source

Acute lysine overload provokes marked striatum injury involving oxidative stress signaling pathways in glutaryl-CoA dehydrogenase deficient mice

Neurochemistry International, 2019
A. U. Amaral, B. Seminotti, Janaína Camacho Silva, F. H. Oliveira, R. Ribeiro, G. Leipnitz, D. Souza, M. Wajner   +7 more
semanticscholar   +1 more source