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.
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Abbreviations
- CAT:
-
Catalase
- DNPH:
-
Dinitrophenylhydrazine
- GAG:
-
Glycosaminoglycans
- GPx:
-
Glutathione peroxidase
- GSH:
-
Glutathione
- IDUA:
-
α-L-iduronidase
- LSD:
-
Lysosomal storage diseases
- MDA:
-
Malondialdehyde
- MPS:
-
Mucopolysaccharidosis
- NADPH:
-
Nicotinamide adenine dinucleotide phosphate diaphorase
- RNS:
-
Reactive nitrogen species
- ROS:
-
Reactive oxygen species
- SOD:
-
Superoxide dismutase
- TBARS:
-
Thiobarbituric acid-reactive substances
- TCA:
-
Trichloroacetic acid
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Acknowledgments
This research was supported by the Millennium Institute of Gene Therapy (CNPq-MCT grant 420036/2005-9), and grants and fellowships from FAPERGS-RS and CAPES. The authors thank Dr. Elizabeth F. Neufeld for her generous gift of Idua +/− mice.
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Reolon, G.K., Reinke, A., de Oliveira, M.R. et al. Alterations in Oxidative Markers in the Cerebellum and Peripheral Organs in MPS I Mice. Cell Mol Neurobiol 29, 443–448 (2009). https://doi.org/10.1007/s10571-008-9335-5
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DOI: https://doi.org/10.1007/s10571-008-9335-5