title: Alterations in oxidative markers in the cerebellum and peripheral organs in MPS I mice.
abstract: Mucopolysaccharidosis type I is a lysosomal storage disease with alterations in several organs. Little is known about the pathways that lead to the pathology. Evidences point oxidative stress on lysosomal storage diseases and mucopolysaccharidosis type I. The aim of the present study was to evaluate oxidative biomarkers on mucopolysaccharidosis type I mice model. We evaluated antioxidant enzymatic activity, protein damage and lipid peroxidation in the forebrain, cerebellum, heart, lung, diaphragm, liver, kidney and spleen. Superoxide dismutase activity was increased on cerebellum, lung, diaphragm, liver and kidney of mucopolysaccharidosis type I mice. Catalase activity was increased on cerebellum, spleen and lung. There was no alteration on glutathione peroxidase activity on any of the analyzed organs. Mucopolysaccharidosis type I mice showed increased carbonyl groups on cerebellum, heart and spleen. There was a decrease of thiobarbituric acid-reactive substances on the cerebellum of mucopolysaccharidosis type I mice. The results indicate a oxidative imbalance in this model. As lysosomes are very susceptible to oxidative damage, leading inclusive to cellular death, and lysosomal storage diseases present several alterations on this organelles, this finding can help to elucidate the cellular damage pathways on mucopolysaccharidosis type I.
authors.affiliation: Department of Pharmacology, Cellular and Molecular Neuropharmacology Research Group, Institute for Basic Health Sciences, and Cancer Research Laboratory, Academic Hospital Research Center, Federal University of Rio Grande do Sul, Porto Alegre, RS, Brazil.
authors.forename: Gustavo Kellermann
authors.initials: GK
authors.lastname: Reolon
authors.forename: Adalisa
authors.initials: A
authors.lastname: Reinke
authors.forename: Marcos Roberto
authors.initials: MR
authors.lastname: de Oliveira
authors.forename: Luisa Macedo
authors.initials: LM
authors.lastname: Braga
authors.forename: Melissa
authors.initials: M
authors.lastname: Camassola
authors.forename: Michael Everton
authors.initials: ME
authors.lastname: Andrades
authors.forename: José Cláudio Fonseca
authors.initials: JC
authors.lastname: Moreira
authors.forename: Nance Beyer
authors.initials: NB
authors.lastname: Nardi
authors.forename: Rafael
authors.initials: R
authors.lastname: Roesler
authors.forename: Felipe
authors.initials: F
authors.lastname: Dal-Pizzol
mesh_terms: D000818:Animals; D000975:Antioxidants / Q000378:metabolism; D015415:Biomarkers / Q000378:metabolism*; D002374:Catalase / Q000378:metabolism; D002531:Cerebellum / Q000378:metabolism*; D004195:Disease Models, Animal; D005979:Glutathione Peroxidase / Q000378:metabolism; D015227:Lipid Peroxidation; D008297:Male; D008315:Malondialdehyde / Q000378:metabolism; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D008059:Mucopolysaccharidosis I / Q000235:genetics / Q000378:metabolism*; D010084:Oxidation-Reduction; D018384:Oxidative Stress; D050050:Protein Carbonylation; D013482:Superoxide Dismutase / Q000378:metabolism; D017392:Thiobarbituric Acid Reactive Substances / Q000378:metabolism; D014018:Tissue Distribution
chemical_list: D000975:Antioxidants; D015415:Biomarkers; D017392:Thiobarbituric Acid Reactive Substances; D008315:Malondialdehyde; D002374:Catalase; D005979:Glutathione Peroxidase; D013482:Superoxide Dismutase