Update on classical and atypical scrapie in sheep and goats

JJ Greenlee - Veterinary pathology, 2019 - journals.sagepub.com
Scrapie is a naturally occurring transmissible spongiform encephalopathy (TSE) or prion
disease of sheep and goats. Scrapie is a protein misfolding disease where the normal 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 …

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 …

Fatal transmissible amyloid encephalopathy: a new type of prion disease associated with lack of prion protein membrane anchoring

B Chesebro, B Race, K Meade-White… - PLoS …, 2010 - journals.plos.org
Prion diseases are fatal neurodegenerative diseases of humans and animals characterized
by gray matter spongiosis and accumulation of aggregated, misfolded, protease-resistant …

Chronic wasting disease in bank voles: characterisation of the shortest incubation time model for prion diseases

MA Di Bari, R Nonno, J Castilla, C D'Agostino… - PLoS …, 2013 - journals.plos.org
In order to assess the susceptibility of bank voles to chronic wasting disease (CWD), we
inoculated voles carrying isoleucine or methionine at codon 109 (Bv109I and Bv109M …

Gerstmann-Sträussler-Scheinker disease subtypes efficiently transmit in bank voles as genuine prion diseases

L Pirisinu, MA Di Bari, C D'Agostino, S Marcon… - Scientific reports, 2016 - nature.com
Abstract Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited neurodegenerative
disorder associated with mutations in the prion protein gene and accumulation of misfolded …

Human prion protein sequence elements impede cross-species chronic wasting disease transmission

TD Kurt, L Jiang, N Fernández-Borges… - The Journal of …, 2015 - Am Soc Clin Investig
Chronic wasting disease (CWD) is a fatal prion disease of North American deer and elk and
poses an unclear risk for transmission to humans. Human exposure to CWD occurs through …

Full restoration of specific infectivity and strain properties from pure mammalian prion protein

CM Burke, DJ Walsh, AD Steele, U Agrimi… - PLoS …, 2019 - journals.plos.org
The protein-only hypothesis predicts that infectious mammalian prions are composed solely
of PrPSc, a misfolded conformer of the normal prion protein, PrPC. However, protein-only …

Cofactor and glycosylation preferences for in vitro prion conversion are predominantly determined by strain conformation

CM Burke, DJ Walsh, KMK Mark, NR Deleault… - PLoS …, 2020 - journals.plos.org
Prion diseases are caused by the misfolding of a host-encoded glycoprotein, PrPC, into a
pathogenic conformer, PrPSc. Infectious prions can exist as different strains, composed of …

Chronic wasting disease (CWD) susceptibility of several North American rodents that are sympatric with cervid CWD epidemics

DM Heisey, NA Mickelsen, JR Schneider… - Journal of …, 2010 - Am Soc Microbiol
Chronic wasting disease (CWD) is a highly contagious always fatal neurodegenerative
disease that is currently known to naturally infect only species of the deer family, Cervidae …