Increased proportions of C1 truncated prion protein protect against cellular M1000 prion infection

Journal of Neuropathology and Experimental Neurology
Victoria A LewisSteven J Collins

Abstract

Prion disease pathogenesis is linked to the cell-associated propagation of misfolded protease-resistant conformers (PrP) of the normal cellular prion protein (PrP). Ongoing PrP expression is the only known absolute requirement for successful prion disease transmission and PrP propagation. Further typifying prion disease is selective neuronal dysfunction and loss, although the precise mechanisms underlying this are undefined. We utilized a single prion strain (M1000) and a range of neuronal and nonneuronal, PrP endogenously expressing and transgenically modified overexpressing cell lines, to evaluate whether PrP glycosylation patterns or constitutive N-terminal cleavage events may be determinants of sustained PrP propagation. Our data demonstrates that relative proportions of full-length and C1 truncated PrP are the most important characteristics influencing susceptibility to sustained M1000 prion infection, supporting PrP alpha-cleavage as a protective event, which may contribute to the selective neuronal vulnerability observed in vivo.

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Citations

Aug 30, 2012·The Journal of Biological Chemistry·Helen M J KlemmVictoria A Lawson
Apr 27, 2012·Molecular Neurodegeneration·Victoria LewisSteven J Collins
Apr 13, 2013·Disease Models & Mechanisms·Layla SinclairCathryn L Haigh
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Aug 25, 2015·Cellular and Molecular Life Sciences : CMLS·Victoria LewisSteven J Collins
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Aug 14, 2020·The Journal of Biological Chemistry·Carola Munoz-MontesinoMichel Dron
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Jan 28, 2022·Cell and Tissue Research·Behnam MohammadiHermann Clemens Altmeppen
Jan 29, 2022·Cell and Tissue Research·Ryan O Walters, Cathryn L Haigh

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