In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: A potential pathophysiological mechanism of striatal damage in this disorder

Molecular Genetics and Metabolism

Volumn 109, Issue 2, 2013, Pages 144-153

  Fernandes, Carolina Gonçalves ^a   da Rosa, Mateus Struecker ^a   Seminotti, Bianca ^a   Pierozan, Paula ^a   Martell, Rafael Wolter ^a   Lagranha, Valeska Lizzi ^a   Busanello, Estela Natacha Brandt ^a   Leipnitz, Guilhian ^a   Wajner, Moacir ^a,b  

^a FEDERAL UNIVERSITY OF RIO GRANDE DO SUL   (Brazil)

^b HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

Author keywords

3 Hydroxy 3 methylglutaric acid; 3 Hydroxy 3 methylglutaric aciduria; 3 Methylglutaric acid; Oxidative stress; Striatum

Indexed keywords

3 METHYLGLUTARIC ACID; ACETYLCYSTEINE; ALPHA TOCOPHEROL; ASCORBIC ACID; CATALASE; DICHLOROFLUORESCEIN; DIZOCILPINE; GLUCOSE 6 PHOSPHATE DEHYDROGENASE; GLUTATHIONE; GLUTATHIONE REDUCTASE; HYDROXYMETHYLGLUTARYL COENZYME A LYASE; MELATONIN; NITRIC OXIDE; REACTIVE OXYGEN METABOLITE; SUPEROXIDE DISMUTASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN DAMAGE; CONTROLLED STUDY; CORPUS STRIATUM; DRUG MECHANISM; ENZYME ACTIVITY; ENZYME DEFICIENCY; HYDROXYMETHYLGLUTARYL COENZYME A LYASE DEFICIENCY; IN VIVO STUDY; LIPID PEROXIDATION; MALE; NONHUMAN; OXIDATIVE STRESS; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PROPHYLAXIS; RAT;

ACETYL-COA C-ACETYLTRANSFERASE; AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; ANTIOXIDANTS; BASAL GANGLIA; CATALASE; DIZOCILPINE MALEATE; GLUTATHIONE; GLUTATHIONE PEROXIDASE; MALE; MALONDIALDEHYDE; MEGLUTOL; OXIDATIVE STRESS; PROTEIN CARBONYLATION; RATS; RATS, WISTAR; RECEPTORS, N-METHYL-D-ASPARTATE; SUPEROXIDE DISMUTASE; VITAMIN E;

RATTUS;

EID: 84878512756     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2013.03.017     Document Type: Article

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