Prions: protein aggregation and infectious diseases

A Aguzzi, AM Calella - Physiological reviews, 2009 - journals.physiology.org
Transmissible spongiform encephalopathies (TSEs) are inevitably lethal neurodegenerative
diseases that affect humans and a large variety of animals. The infectious agent responsible …

Chronic wasting disease in Europe: new strains on the horizon

MA Tranulis, D Gavier-Widén, J Våge… - Acta Veterinaria …, 2021 - Springer
Prion diseases are fatal neurodegenerative disorders with known natural occurrence in
humans and a few other mammalian species. The diseases are experimentally …

Insights into prion strains and neurotoxicity

A Aguzzi, M Heikenwalder… - Nature reviews Molecular …, 2007 - nature.com
Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that
are caused by prions and affect humans and many animal species. It is now widely accepted …

Full atomistic model of prion structure and conversion

G Spagnolli, M Rigoli, S Orioli, AM Sevillano… - PLoS …, 2019 - journals.plos.org
Prions are unusual protein assemblies that propagate their conformationally-encoded
information in absence of nucleic acids. The first prion identified, the scrapie isoform (PrPSc) …

Molecular mechanisms of prion pathogenesis

A Aguzzi, C Sigurdson… - Annu. Rev. Pathol. Mech …, 2008 - annualreviews.org
Prion diseases are infectious neurodegenerative diseases occurring in humans and animals
with an invariably lethal outcome. One fundamental mechanistic event in prion diseases is …

Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD

S Hannaoui, I Zemlyankina, SC Chang, MI Arifin… - Acta …, 2022 - Springer
Prions cause infectious and fatal neurodegenerative diseases in mammals. Chronic wasting
disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed …

Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in …

P Parchi, L De Boni, D Saverioni, ML Cohen… - Acta …, 2012 - Springer
The current classification of human sporadic prion diseases recognizes six major phenotypic
subtypes with distinctive clinicopathological features, which largely correlate at the …

Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification

P Parchi, R Strammiello, S Notari, A Giese… - Acta …, 2009 - Springer
Six subtypes of sporadic Creutzfeldt–Jakob disease with distinctive clinico-pathological
features have been identified largely based on two types of the abnormal prion protein, PrP …

Bank vole prion protein as an apparently universal substrate for RT-QuIC-based detection and discrimination of prion strains

CD Orrú, BR Groveman, LD Raymond… - PLoS …, 2015 - journals.plos.org
Prions propagate as multiple strains in a wide variety of mammalian species. The detection
of all such strains by a single ultrasensitive assay such as Real Time Quaking-induced …

Pathogenesis of prion diseases: current status and future outlook

A Aguzzi, M Heikenwalder - Nature Reviews Microbiology, 2006 - nature.com
The prion, a conformational variant of a host protein, is the infectious particle responsible for
transmissible spongiform encephalopathy (TSE), a fatal neurodegenerative disease of …