Neurodegenerative diseases: expanding the prion concept

LC Walker, M Jucker - Annual review of neuroscience, 2015 - annualreviews.org
The prion paradigm has emerged as a unifying molecular principle for the pathogenesis of
many age-related neurodegenerative diseases. This paradigm holds that a fundamental …

The prion-like properties of amyloid-β assemblies: implications for Alzheimer's disease

LC Walker, J Schelle, M Jucker - Cold Spring …, 2016 - perspectivesinmedicine.cshlp.org
Since the discovery that prion diseases can be transmitted to experimental animals by
inoculation with afflicted brain matter, researchers have speculated that the brains of …

Prion-like mechanisms in Alzheimer disease

LC Walker - Handbook of clinical neurology, 2018 - Elsevier
Senile plaques and neurofibrillary tangles are the principal histopathologic hallmarks of
Alzheimer disease. The essential constituents of these lesions are structurally abnormal …

Understanding the key features of the spontaneous formation of bona fide prions through a novel methodology that enables their swift and consistent generation

H Eraña, CM Díaz-Domínguez, JM Charco… - Acta neuropathologica …, 2023 - Springer
Among transmissible spongiform encephalopathies or prion diseases affecting humans,
sporadic forms such as sporadic Creutzfeldt–Jakob disease are the vast majority. Unlike …

Development of a new largely scalable in vitro prion propagation method for the production of infectious recombinant prions for high resolution structural studies

H Eraña, JM Charco, MA Di Bari… - PLoS …, 2019 - journals.plos.org
The resolution of the three-dimensional structure of infectious prions at the atomic level is
pivotal to understand the pathobiology of Transmissible Spongiform Encephalopathies …

Isolation of infectious, non-fibrillar and oligomeric prions from a genetic prion disease

I Vanni, L Pirisinu, C Acevedo-Morantes… - Brain, 2020 - academic.oup.com
Prions are transmissible agents causing lethal neurodegenerative diseases that are
composed of aggregates of misfolded cellular prion protein (PrPSc). Despite non-fibrillar …

Heparan sulfate and heparin promote faithful prion replication in vitro by binding to normal and abnormal prion proteins in protein misfolding cyclic amplification

M Imamura, N Tabeta, N Kato, Y Matsuura… - Journal of Biological …, 2016 - ASBMB
The precise mechanism underlying the conversion of normal prion protein (PrP C) into
abnormal prion protein (PrP Sc) remains unclear. Protein misfolding cyclic amplification …

Treatment with a non-toxic, self-replicating anti-prion delays or prevents prion disease in vivo

R Diaz-Espinoza, R Morales, L Concha-Marambio… - Molecular …, 2018 - nature.com
Transmissible spongiform encephalopathies (TSEs) are fatal neurological disorders caused
by prions, which are composed of a misfolded protein (PrP Sc) that self-propagates in the …

[HTML][HTML] REST alleviates neurotoxic prion peptide-induced synaptic abnormalities, neurofibrillary degeneration and neuronal death partially via LRP6-mediated Wnt-β …

Z Song, T Zhu, X Zhou, P Barrow, W Yang, Y Cui… - Oncotarget, 2016 - ncbi.nlm.nih.gov
Prion diseases are a group of infectious neurodegenerative diseases characterized by
multiple neuropathological hallmarks including synaptic damage, spongiform degeneration …

Lithium alleviates neurotoxic prion peptide-induced synaptic damage and neuronal death partially by the upregulation of nuclear target REST and the restoration of …

Z Song, W Yang, X Zhou, L Yang, D Zhao - Neuropharmacology, 2017 - Elsevier
Prion diseases are a group of infectious neurodegenerative diseases characterized by
multiple neuropathological hallmarks, including accumulation of PrP Sc, synaptic damage …