A hypothesis on prion disorders: are infectious, inherited, and sporadic causes so distinct?

Brain Research Bulletin
F FornaiA Paparelli

Abstract

Prion diseases include a group of either sporadic, inherited or infectious disorders characterized by spongiform neurodegeneration and reactive glyosis in several brain regions. Whatever the origin, the neuropathological hallmark of prion diseases is the presence of brain aggregates containing an altered isoform of a cellular protein, named prion protein. Recent findings show the potential toxicity of the normal cellular prion protein, which occurs when its physiological metabolism is altered. In particular, several studies demonstrate that accumulation of the prion protein in the cytosol can be a consequence of an increased amount of misfolded prion proteins, a derangement of the correct protein trafficking or a reduced activity of the ubiquitin-proteasome system. The same effects can be a consequence of a mutation in the gene coding for the prion protein. In all these conditions, one assists to accumulation and self-replication of insoluble prion proteins which leads to a severe disease resembling what observed following typical "prion infections". This article provides an opinion aimed at reconciling the classic Prusiner's theory concerning the "prion concepts" with the present knowledge arising from experimental studies on ...Continue Reading

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Citations

Jul 18, 2009·Muscle & Nerve·Livia PasqualiFrancesco Fornai
Sep 15, 2014·Journal of Neural Transmission·Filippo Sean GiorgiFrancesco Fornai
Aug 5, 2011·Prion·Gianfranco NataleFrancesco Fornai
Sep 16, 2008·Biochimica Et Biophysica Acta·Brandi R WhatleyLih-Shen Chin
Apr 9, 2008·Physiological Reviews·Rafael LindenRicardo R Brentani
Apr 25, 2020·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Rawiah A AlsiaryAbdul-Hamid Emwas
Oct 18, 2019·International Journal of Molecular Sciences·Larisa RyskalinFrancesco Fornai
Feb 6, 2021·Scientific Reports·Sudheer Babu SangeethamErvin Welker

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