title: The effect of metal imbalances on scrapie neurodegeneration.
abstract: Environmental exposure to metal appears to enhance susceptibility to Transmissible Spongiform Encephalopathies (TSEs); however, published data are not conclusive. The current study focuses on assessing the effects of copper depletion and/or manganese enhancement in the diet on susceptibility to Scrapie and this disease progression. The degree of spongiosis was the highest in the animals that received a copper- depleted diet. These observations suggest that this diet contributes to the Scrapie lesions and to the worsening of the condition in animals that have been inoculated with Scrapie. The highest intensities of GFAP immunostaining were also associated with the copper- depleted diet. Dietary supplementation with manganese had a negative effect on neuronal counts. In conclusion, this study demonstrates that certain environmental factors may aggravate neuropathological Scrapie lesions. This is consistent with reports from other neurodegenerative diseases where some metalloenzymes play a pivotal protector role against the oxidative stress associated with pathogenesis.
authors.affiliation: Research Centre for Prion Diseases, University of Zaragoza, Spain.
authors.forename: P
authors.initials: P
authors.lastname: Hortells
authors.forename: E
authors.initials: E
authors.lastname: Monleón
authors.forename: C
authors.initials: C
authors.lastname: Acín
authors.forename: A
authors.initials: A
authors.lastname: Vargas
authors.forename: V
authors.initials: V
authors.lastname: Vasseur
authors.forename: A
authors.initials: A
authors.lastname: Salomon
authors.forename: B
authors.initials: B
authors.lastname: Ryffel
authors.forename: J Y
authors.initials: JY
authors.lastname: Cesbron
authors.forename: J J
authors.initials: JJ
authors.lastname: Badiola
authors.forename: M
authors.initials: M
authors.lastname: Monzón
mesh_terms: D000821:Animal Feed; D000824:Animal Nutritional Physiological Phenomena; D000818:Animals; D001921:Brain / Q000473:pathology; D003300:Copper / Q000172:deficiency* / Q000378:metabolism; D004032:Diet; D019587:Dietary Supplements; D008345:Manganese / Q000494:pharmacology*; D008670:Metals / Q000378:metabolism* / Q000494:pharmacology; D051379:Mice; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics / Q000378:metabolism; D012608:Scrapie / Q000378:metabolism* / Q000473:pathology
chemical_list: D008670:Metals; D000072002:Prion Proteins; D011328:Prions; C512657:Prnp protein, mouse; D008345:Manganese; D003300:Copper