... degradation of p53 amyloids by proteasomal machinery in cells can further generate secondary infectious protein aggregates (seeds), which can aid in cell-to-cell transmission and the acquisition of inheritable p53 prion transmission (Halfmann et al., 2012 ; Liebman and Chernoff, 2012 ; Wickner et al., 2015...

... strategies for amyloid disorders. Amyloid Autophagy Disaggregase Prion Neurodegeneration National Institutes of Health 10.13039/100000002 T32GM008076 R01GM099836 R21NS090205 R21NS102687 Life Extension Foundation Sanofi 10.13039/100004339 Office...

...Ewan West; Craig Osborne; Clive Bate ABSTRACT Cholesterol is required for the formation and function of some signalling platforms. In synaptosomes, amyloid-β (Aβ) oligomers, the causative agent in Alzheimer's disease, bind to cellular prion proteins (PrP C) resulting in increased cholesterol...

...Kensuke Kataoka; Kazufumi Mochizuki ABSTRACT Regulated aggregations of prion and prion-like proteins play physiological roles in various biological processes. However, their structural roles in the nucleus are poorly understood. Here, we show that the prion-like protein Jub6p is involved...

...Yang-In Yim; Bum-Chan Park; Rajgopal Yadavalli; Xiaohong Zhao; Evan Eisenberg; Lois E. Greene ABSTRACT The conversion of the properly folded prion protein, PrPc, to its misfolded amyloid form, PrPsc, occurs as the two proteins traffic along the endocytic pathway and PrPc is exposed to PrPsc...

...Mimi Cushman; Brian S. Johnson; Oliver D. King; Aaron D. Gitler; James Shorter Prions are proteins that access self-templating amyloid forms, which confer phenotypic changes that can spread from individual to individual within or between species. These infectious phenotypes can be beneficial...

...Young-Shin Kang; Xiaohong Zhao; Jenna Lovaas; Evan Eisenberg; Lois E. Greene To understand the role of clathrin-mediated endocytosis in the internalization of normal cellular prion protein (PrP c) in neuronal cells, N2a cells were depleted of clathrin by RNA interference. PrP c internalization via...

...Cathryn L. Haigh; Simon C. Drew; Martin P. Boland; Colin L. Masters; Kevin J. Barnham; Victoria A. Lawson; Steven J. Collins Beta-cleavage of the neurodegenerative disease-associated prion protein (PrP) protects cells from death induced by oxidative insults. The beta-cleavage event produces two...

...Kyung-Jin Lee; Antony Panzera; David Rogawski; Lois E. Greene; Evan Eisenberg The effect of normal cellular prion protein (PrP C) on abnormal protein aggregation was examined by transfecting huntingtin fragments (Htt) into SN56 neuronal-derived cells depleted of PrP C by RNA interference. PrP C...

...Max Nunziante; Claudia Kehler; Elke Maas; Matthias U. Kassack; Martin Groschup; Hermann M. Schätzl The conversion of the cellular prion protein (PrP c) into a pathogenic isoform (PrP Sc) is one of the underlying events in the pathogenesis of the fatal transmissible spongiform encephalopathies...

...Carole Crozet; Yea-Lih Lin; Clément Mettling; Chantal Mourton-Gilles; Pierre Corbeau; Sylvain Lehmann; Véronique Perrier Currently, there is no treatment to cure transmissible spongiform encephalopathies. By taking advantage of the `prion-resistant' polymorphisms Q171R and E219K that naturally...

...Axelle Balguerie; Suzana Dos Reis; Bénédicte Coulary-Salin; Stéphane Chaignepain; Martine Sabourin; Jean-Marie Schmitter; Sven J. Saupe The [Het-s] prion of the fungus Podospora anserina propagates as a self-perpetuating amyloid form of the HET-s protein. This protein triggers a cell death...

...Alain Mangé; Carole Crozet; Sylvain Lehmann; Florence Béranger Prion diseases are fatal transmissible neurodegenerative disorders characterized by the accumulation of an abnormally folded isoform of the cellular prion protein (PrP C) denoted PrP Sc . Recently, wild-type and pathogenic PrP mutants...

...Nathalie Daude; Mathieu Marella; Joëlle Chabry Development of transmissible spongiform encephalopathies (TSEs)pathogenesis requires the presence of both the normal host prion protein(PrP-sen) and the abnormal pathological proteinase-K resistant isoform(PrP-res). PrP-res forms highly insoluble...