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 …
numerous mammalian species, including humans. The existence of heritable phenotypes of …
Pathogenic prion structures at high resolution
B Caughey, HG Standke, E Artikis, F Hoyt… - PLoS …, 2022 - journals.plos.org
Numerous proteins are known to form ordered, self-propagating aggregates as underlying
causes of neurodegeneration in proteinopathies such as Alzheimer's, Parkinson's, and prion …
causes of neurodegeneration in proteinopathies such as Alzheimer's, Parkinson's, and prion …
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 …
of PrPSc, a misfolded conformer of the normal prion protein, PrPC. However, protein-only …
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 …
pivotal to understand the pathobiology of Transmissible Spongiform Encephalopathies …
The prion 2018 round tables (I): the structure of PrPSc
Understanding the structure of PrPSc is without doubt a sine qua non to understand not only
PrPSc propagation, but also critical features of that process such as the strain phenomenon …
PrPSc propagation, but also critical features of that process such as the strain phenomenon …
The cellular and pathologic prion protein
AC Gill, AR Castle - Handbook of clinical neurology, 2018 - Elsevier
The cellular prion protein, PrP C, is a small, cell surface glycoprotein with a function that is
currently somewhat ill defined. It is also the key molecule involved in the family of …
currently somewhat ill defined. It is also the key molecule involved in the family of …
Yeast prions compared to functional prions and amyloids
RB Wickner, HK Edskes, M Son, EE Bezsonov… - Journal of molecular …, 2018 - Elsevier
Saccharomyces cerevisiae is an occasional host to an array of prions, most based on self-
propagating, self-templating amyloid filaments of a normally soluble protein.[URE3] is a …
propagating, self-templating amyloid filaments of a normally soluble protein.[URE3] is a …
Structural attributes of mammalian prion infectivity: Insights from studies with synthetic prions
Prion diseases are neurodegenerative disorders that affect many mammalian species.
Mammalian prion proteins (PrPs) can misfold into many different aggregates. However, only …
Mammalian prion proteins (PrPs) can misfold into many different aggregates. However, only …
Transmissibility versus pathogenicity of self-propagating protein aggregates
The prion-like spreading and accumulation of specific protein aggregates appear to be
central to the pathogenesis of many human diseases, including Alzheimer's and …
central to the pathogenesis of many human diseases, including Alzheimer's and …
Immunotherapy against prion disease
Y Ma, J Ma - Pathogens, 2020 - mdpi.com
The term “prion disease” encompasses a group of neurodegenerative diseases affecting
both humans and animals. Currently, there is no effective therapy and all forms of prion …
both humans and animals. Currently, there is no effective therapy and all forms of prion …