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Open Life Sciences

formerly Central European Journal of Biology

Editor-in-Chief: Ratajczak, Mariusz

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Volume 5, Issue 1


Volume 10 (2015)

ACAT-1, Cav-1 and PrP expression in scrapie susceptible and resistant sheep

Cristina Orrù / Claudia Abete / M. Dolores Cannas / Claudia Mulas / Claudia Norfo / Antonella Mandas / Sarah Vascellari / Paolo Colla / Sandra Dessì / Alessandra Pani
Published Online: 2010-01-30 | DOI: https://doi.org/10.2478/s11535-009-0076-3


Scrapie is a prion disease for which no means of ante-mortem diagnosis is available. We recently found a relationship between cell susceptibility to scrapie and altered cholesterol homeostasis. In brains and in skin fibroblasts and peripheral blood mononuclear cells from healthy and scrapie-affected sheep carrying a scrapie-susceptible genotype, the levels of cholesterol esters were consistently higher than in tissues and cultures derived from animals with a scrapie-resistant genotype. Here we show that intracellular accumulation of cholesterol esters (CE) in fibroblasts derived from scrapie-susceptible sheep was accompanied by parallel alterations in the expression level of acyl-coenzymeA: cholesterol-acyltransferase (ACAT1) and caveolin-1 (Cav-1) that are involved in the pathways leading to intracellular cholesterol esterification and trafficking. Comparative analysis of cellular prion protein (PrPc) mRNA, showed an higher expression level in cells from animals carrying a susceptible genotype, with or without Scrapie. These data suggest that CE accumulation in peripheral cells, together with the altered expression of some proteins implicated in intracellular cholesterol homeostasis, might serve to identify a distinctive lipid metabolic profile associated with increased susceptibility to develop prion disease following infection.

Keywords: Scrapie; Prion diseases; Cholesterol homeostasis; Cholesterol esters

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About the article

Published Online: 2010-01-30

Published in Print: 2010-02-01

Citation Information: Open Life Sciences, Volume 5, Issue 1, Pages 31–37, ISSN (Online) 2391-5412, DOI: https://doi.org/10.2478/s11535-009-0076-3.

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© 2010 Versita Warsaw. This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License. BY-NC-ND 3.0

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