Transmissible spongiform encephalopathies (TSEs) are inevitably lethal neurodegenerative
diseases that affect humans and a large variety of animals. The infectious agent responsible …

Prion diseases are a group of inevitably fatal neurodegenerative disorders affecting
numerous mammalian species, including humans. The existence of heritable phenotypes of …

Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-
Jakob disease in humans, scrapie in small ruminants, and bovine spongiform …

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 …

The NMR structure of the horse (Equus caballus) cellular prion protein at 25° C exhibits the
typical PrPC [cellular form of prion protein (PrP)] global architecture, but in contrast to most …

In prion diseases, the species barrier limits the transmission of prions from one species to
another. However, cross-species prion transmission is remarkably efficient in bank voles …

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 …

Prions are self-propagating protein conformers that cause a variety of neurodegenerative
disorders in humans and animals. Mouse models have played key roles in deciphering the …

Most cases of human prion disease arise due to spontaneous misfolding of WT or mutant
prion protein, yet recapitulating this event in animal models has proven challenging. It …

Abstract Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited neurodegenerative
disorder associated with mutations in the prion protein gene and accumulation of misfolded …