Oxidative Stress, Disrupted Energy Metabolism, and Altered Signaling Pathways in Glutaryl-CoA Dehydrogenase Knockout Mice: Potential Implications of Quinolinic Acid Toxicity in the Neuropathology of Glutaric Acidemia Type I

We investigated the effects of an acute intrastriatal QUIN administration on cellular redox and bioenergetics homeostasis, as well as on important signaling pathways in the striatum of wild-type ( Gcdh +/+ , WT) and knockout mice for glutaryl-CoA dehydrogenase ( Gcdh −/− ) fed a high lysine (Lys, 4....

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Veröffentlicht in:	Molecular neurobiology 2016-11, Vol.53 (9), p.6459-6475
Hauptverfasser:	Seminotti, Bianca, Amaral, Alexandre Umpierrez, Ribeiro, Rafael Teixeira, Rodrigues, Marília Danyelle Nunes, Colín-González, Ana Laura, Leipnitz, Guilhian, Santamaría, Abel, Wajner, Moacir
Format:	Artikel
Sprache:	eng
Schlagworte:	Amino Acid Metabolism, Inborn Errors - enzymology Amino Acid Metabolism, Inborn Errors - metabolism Amino Acid Metabolism, Inborn Errors - pathology Animals Antioxidants - metabolism Bioenergetics Biomedical and Life Sciences Biomedicine Brain Diseases, Metabolic - enzymology Brain Diseases, Metabolic - metabolism Brain Diseases, Metabolic - pathology Cell Biology Diet Electron Transport Complex IV - metabolism Energy Metabolism - drug effects Extracellular Signal-Regulated MAP Kinases - metabolism Fluoresceins - metabolism Glutaryl-CoA Dehydrogenase - deficiency Glutaryl-CoA Dehydrogenase - metabolism Glutathione - metabolism Glutathione Peroxidase - metabolism Homeostasis Inflammation Lactates - metabolism Lipid Peroxidation - drug effects Malondialdehyde - metabolism Mice, Knockout Models, Biological Neostriatum - drug effects Neostriatum - metabolism Neostriatum - pathology Neurobiology Neurology Neurosciences NF-E2-Related Factor 2 - metabolism NF-kappa B - metabolism Nitrates - metabolism Nitrites - metabolism Oxidation-Reduction Oxidative stress Oxidative Stress - drug effects Proto-Oncogene Proteins c-akt - metabolism Quinolinic Acid - administration & dosage Quinolinic Acid - toxicity Signal transduction Signal Transduction - drug effects Sulfhydryl Compounds - metabolism Superoxide Dismutase - metabolism
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