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 …

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 …

Cellular and molecular mechanisms of prion disease

CJ Sigurdson, JC Bartz, M Glatzel - Annual Review of Pathology …, 2019 - annualreviews.org
Prion diseases are rapidly progressive, incurable neurodegenerative disorders caused by
misfolded, aggregated proteins known as prions, which are uniquely infectious. Remarkably …

Prion agent diversity and species barrier

V Béringue, JL Vilotte, H Laude - Veterinary research, 2008 - hal.science
Mammalian prions are the infectious agents responsible for transmissible spongiform
encephalopathies (TSE), a group of fatal, neurodegenerative diseases, affecting both …

Evidence that bank vole PrP is a universal acceptor for prions

JC Watts, K Giles, S Patel, A Oehler… - PLoS …, 2014 - journals.plos.org
Bank voles are uniquely susceptible to a wide range of prion strains isolated from many
different species. To determine if this enhanced susceptibility to interspecies prion …

Prion strains: shining new light on old concepts

AJ Block, JC Bartz - Cell and Tissue Research, 2023 - Springer
Prion diseases are a group of inevitably fatal neurodegenerative disorders affecting
numerous mammalian species, including humans. The existence of heritable phenotypes of …

Deer prion proteins modulate the emergence and adaptation of chronic wasting disease strains

C Duque Velásquez, C Kim, A Herbst, N Daude… - Journal of …, 2015 - Am Soc Microbiol
Transmission of chronic wasting disease (CWD) between cervids is influenced by the
primary structure of the host cellular prion protein (PrPC). In white-tailed deer, PRNP alleles …

Evidence for preexisting prion substrain diversity in a biologically cloned prion strain

T Gunnels, RA Shikiya, TC York, AJ Block… - PLoS …, 2023 - journals.plos.org
Prion diseases are a group of inevitably fatal neurodegenerative disorders affecting
numerous mammalian species, including Sapiens. Prions are composed of PrPSc, the …

Prion strain diversity

JC Bartz - Cold Spring Harbor perspectives in …, 2016 - perspectivesinmedicine.cshlp.org
Prion diseases affect a wide range of mammal species and are caused by a misfolded self-
propagating isoform (PrPSc) of the normal prion protein (PrPC). Distinct strains of prions …

Multifaceted role of sialylation in prion diseases

IV Baskakov, E Katorcha - Frontiers in Neuroscience, 2016 - frontiersin.org
Mammalian prion or PrPSc is a proteinaceous infectious agent that consists of a misfolded,
self-replicating state of a sialoglycoprotein called the prion protein, or PrPC. Sialylation of …