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N\u2010butyl\u2010deoxynojirimycin is the only approved drug for patients with NPC disease. It inhibits glycosphingolipid synthesis, therefore reducing intracellular lipid storage. Although the mechanisms underlying the neurologic damage in the NPC disease are not yet well established, in vitro and in vivo studies suggest an involvement of reactive species in the pathophysiology of this disease. In this work we aimed to evaluate parameters of lipid and protein oxidation, measured by thiobarbituric acid\u2010reactive species (TBA\u2010RS) and protein carbonyl formation, respectively, as well as the enzymatic and non\u2010enzymatic antioxidant defenses in plasma, erythrocytes and fibroblasts from NPC1 patients, at diagnosis and during treatment with N\u2010butyl\u2010deoxynojirimycin. We found a significant increase of TBA\u2010RS in plasma and fibroblasts, as well as increased protein carbonyl formation and decreased total antioxidant status (TAS) in plasma of untreated NPC1 patients as compared to the control group. In addition, erythrocyte glutathione peroxidase (GSH\u2010Px) activity was increased, whereas CAT and SOD activities were normal in these patients. We also observed that patients treated with N\u2010butyl\u2010deoxynojirimycin normalized plasma TBA\u2010RS and TAS, as well as erythrocyte GSH\u2010Px activity. Taken together, the present data indicate that oxidative stress is increased in patients with NPC1 disease and that treatment with N\u2010butyl\u2010deoxynojirimycin is able to confer protection against this pathological process.<\/jats:p>","DOI":"10.1016\/j.ijdevneu.2012.07.002","type":"journal-article","created":{"date-parts":[[2012,7,20]],"date-time":"2012-07-20T22:02:30Z","timestamp":1342821750000},"page":"439-444","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["Oxidative stress in Niemann\u2010Pick type C patients: a protective role of N\u2010butyl\u2010deoxynojirimycin therapy"],"prefix":"10.1002","volume":"30","author":[{"given":"Graziela S.","family":"Ribas","sequence":"first","affiliation":[{"name":"Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias Farmac\u00eauticas UFRGS Ipiranga 2752 Porto Alegre RS 90610\u2010000 Brazil"},{"name":"Servi\u00e7o de Gen\u00e9tica M\u00e9dica HCPA Ramiro Barcelos 2350 Porto Alegre RS 90035\u2010903 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo","family":"Pires","sequence":"additional","affiliation":[{"name":"Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Gen\u00e9tica e Toxicologia Aplicada ULBRA Av. 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