title: Oxidative stress in a phenylketonuria animal model.
abstract: Oxidative stress is seen in various metabolic disorders for unknown reasons. Oxidative stress is defined as an imbalance between pro-oxidant and antioxidant status in favor of the former. This study investigated whether oxidative stress exists in phenylketonuria (PKU) using the BTBR-Pah(enu2) animal model for PKU. Animals (14-24 weeks old) were sacrificed and brain and red blood cells (RBCs) were obtained aseptically. The lipid peroxidation by-product, evaluated as malondialdehyde (MDA), was significantly higher in the brains and RBCs of PKU animals (n = 6) than in controls (n = 6). Glutathione/glutathione disulfide, a good indicator for tissue thiol status, was significantly decreased both in the brains and RBCs. Some antioxidant enzymes were also analyzed in RBCs, including glucose-6-phosphate dehydrogenase (G6PD), which provides the RBC's main reducing power, reduced nicotinamide adenine dinucleotide phosphate (NADPH), and catalase detoxifies H2O2 by catalyzing its reduction to O2 and H2O. Both catalase and G6PD were significantly increased in the RBCs of PKU animals.
authors.affiliation: University of Missouri-Rolla, Department of Chemistry, 65409, USA. nercal@umr.edu
authors.forename: Nuran
authors.initials: N
authors.lastname: Ercal
authors.forename: Nukhet
authors.initials: N
authors.lastname: Aykin-Burns
authors.forename: Hande
authors.initials: H
authors.lastname: Gurer-Orhan
authors.forename: J David
authors.initials: JD
authors.lastname: McDonald
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism; D002374:Catalase / Q000378:metabolism; D002851:Chromatography, High Pressure Liquid; D004195:Disease Models, Animal; D004912:Erythrocytes / Q000378:metabolism; D005954:Glucosephosphate Dehydrogenase / Q000378:metabolism; D005978:Glutathione / Q000378:metabolism; D006861:Hydrogen Peroxide / Q000378:metabolism; D015227:Lipid Peroxidation; D008315:Malondialdehyde / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D009249:NADP / Q000378:metabolism; D010084:Oxidation-Reduction; D018384:Oxidative Stress; D010100:Oxygen / Q000378:metabolism; D010649:Phenylalanine / Q000378:metabolism; D010661:Phenylketonurias / Q000378:metabolism*
chemical_list: D010649:Phenylalanine; D008315:Malondialdehyde; D009249:NADP; D006861:Hydrogen Peroxide; D005954:Glucosephosphate Dehydrogenase; D002374:Catalase; D005978:Glutathione; D010100:Oxygen