Prions, prionoids and protein misfolding disorders

C Scheckel, A Aguzzi - Nature Reviews Genetics, 2018 - nature.com
Prion diseases are progressive, incurable and fatal neurodegenerative conditions. The term
'prion'was first nominated to express the revolutionary concept that a protein could be …

Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases

JL Guo, VMY Lee - Nature medicine, 2014 - nature.com
A common feature of many neurodegenerative diseases is the deposition of β-sheet-rich
amyloid aggregates formed by proteins specific to these diseases. These protein aggregates …

A single-cell map of antisense oligonucleotide activity in the brain

MA Mortberg, JE Gentile, NM Nadaf… - Nucleic Acids …, 2023 - academic.oup.com
Antisense oligonucleotides (ASOs) dosed into cerebrospinal fluid (CSF) distribute broadly
throughout the central nervous system (CNS). By modulating RNA, they hold the promise of …

Brainwide silencing of prion protein by AAV-mediated delivery of an engineered compact epigenetic editor

EN Neumann, TM Bertozzi, E Wu, F Serack, JW Harvey… - Science, 2024 - science.org
Prion disease is caused by misfolding of the prion protein (PrP) into pathogenic self-
propagating conformations, leading to rapid-onset dementia and death. However …

Follicular dendritic cells: dynamic antigen libraries

BA Heesters, RC Myers, MC Carroll - Nature Reviews Immunology, 2014 - nature.com
Follicular dendritic cells (FDCs) are essential for high-affinity antibody production and for the
development of B cell memory. Historically, FDCs have been characterized as' …

Protein aggregation diseases: pathogenicity and therapeutic perspectives

A Aguzzi, T O'connor - Nature reviews Drug discovery, 2010 - nature.com
A growing number of diseases seem to be associated with inappropriate deposition of
protein aggregates. Some of these diseases—such as Alzheimer's disease and systemic …

PROTOFIBRILS, PORES, FIBRILS, AND NEURODEGENERATION: Separating the Responsible Protein Aggregates from The Innocent Bystanders

B Caughey, PT Lansbury Jr - Annual review of neuroscience, 2003 - annualreviews.org
▪ Abstract Many neurodegenerative diseases, including Alzheimer's and Parkinson's and the
transmissible spongiform encephalopathies (prion diseases), are characterized at autopsy …

Prion diseases of humans and animals: their causes and molecular basis

J Collinge - Annual review of neuroscience, 2001 - annualreviews.org
▪ Abstract Prion diseases are transmissible neurodegenerative conditions that include
Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) …

A general model of prion strains and their pathogenicity

J Collinge, AR Clarke - Science, 2007 - science.org
Prions are lethal mammalian pathogens composed of aggregated conformational isomers of
a host-encoded glycoprotein and which appear to lack nucleic acids. Their unique biology …

Mammalian prions and their wider relevance in neurodegenerative diseases

J Collinge - Nature, 2016 - nature.com
Prions are notorious protein-only infectious agents that cause invariably fatal brain diseases
following silent incubation periods that can span a lifetime. These diseases can arise …