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Cellular prion protein neuroprotective function: implications in prion diseases

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Abstract

Prion protein can display two conformations: a normal cellular conformation (PrP) and a pathological conformation associated with prion diseases (PrPSc). Three complementary strategies are used by researchers investigating how PrP is involved in the pathogenesis of prion diseases: elucidation of the normal function of PrP, determination of how PrPSc is toxic to neurons, and unraveling the mechanism for the conversion of PrP to PrPSc. We review the normal function of PrP as an antioxidant and an antiapoptotic protein in vivo and in vitro. This review also addresses contrasting evidence that PrP is cytotoxic. Finally, we discuss the implication of the neuroprotective role of PrP in prion diseases.

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Abbreviations

BH2 :

Bcl-2 homology domain

ERK :

Extracellular regulated kinase

GPI :

Glycophosphatidylinositol

PrP :

Prion protein

TNF :

Tumor necrosis factor

PrP Sc :

PrP scrapie

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  1. Xavier Roucou

    Present address: Department of Biochemistry, Faculty of Medicine, University of Sherbrooke, 3001 12E Avenue Nord, Fleurimont, QC, J1H 5N4, Canada

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  1. Bloomfield Center for Research in Aging, Lady Davis Institute for Medical Research, 3755 Ch. Cote Ste-Catherine, Montreal, QC, H3T 1E2, Canada

    Xavier Roucou & Andréa C. LeBlanc

  2. Department of Neurology and Neurosurgery, McGill University, Montreal, QC, Canada

    Andréa C. LeBlanc

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Roucou, X., LeBlanc, A.C. Cellular prion protein neuroprotective function: implications in prion diseases. J Mol Med 83, 3–11 (2005). https://doi.org/10.1007/s00109-004-0605-5

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