The function of the cellular prion protein in health and disease

JC Watts, MEC Bourkas, H Arshad - Acta neuropathologica, 2018 - Springer
The essential role of the cellular prion protein (PrP C) in prion disorders such as Creutzfeldt–
Jakob disease is well documented. Moreover, evidence is accumulating that PrP C may act …

Human prion disease: molecular pathogenesis, and possible therapeutic targets and strategies

S Baiardi, A Mammana, S Capellari… - Expert Opinion on …, 2023 - Taylor & Francis
Introduction Human prion diseases are heterogeneous, and often rapidly progressive,
transmissible neurodegenerative disorders associated with misfolded prion protein (PrP) …

Prion disease: experimental models and reality

S Brandner, Z Jaunmuktane - Acta neuropathologica, 2017 - Springer
The understanding of the pathogenesis and mechanisms of diseases requires a
multidisciplinary approach, involving clinical observation, correlation to pathological …

The N-terminus of the prion protein is a toxic effector regulated by the C-terminus

B Wu, AJ McDonald, K Markham, CB Rich, KP McHugh… - Elife, 2017 - elifesciences.org
PrPC, the cellular isoform of the prion protein, serves to transduce the neurotoxic effects of
PrPSc, the infectious isoform, but how this occurs is mysterious. Here, using a combination …

Prion protein as a toxic acceptor of amyloid-β oligomers

SA Purro, AJ Nicoll, J Collinge - Biological psychiatry, 2018 - Elsevier
The initial report that cellular prion protein (PrP C) mediates toxicity of amyloid-β species
linked to Alzheimer's disease was initially treated with scepticism, but growing evidence …

Toward therapy of human prion diseases

A Aguzzi, AKK Lakkaraju… - Annual review of …, 2018 - annualreviews.org
Three decades after the discovery of prions as the cause of Creutzfeldt-Jakob disease and
other transmissible spongiform encephalopathies, we are still nowhere close to finding an …

Mechanisms of prion-induced toxicity

RCC Mercer, DA Harris - Cell and Tissue Research, 2023 - Springer
Prion diseases are devastating neurodegenerative diseases caused by the structural
conversion of the normally benign prion protein (PrPC) to an infectious, disease-associated …

Vectored immunotherapeutics for infectious diseases: Can rAAVs be the game changers for fighting transmissible pathogens?

W Zhan, M Muhuri, PWL Tai, G Gao - Frontiers in immunology, 2021 - frontiersin.org
Conventional vaccinations and immunotherapies have encountered major roadblocks in
preventing infectious diseases like HIV, influenza, and malaria. These challenges are due to …

Alzheimer's disease Aβ assemblies mediating rapid disruption of synaptic plasticity and memory

I Klyubin, WK Cullen, NW Hu, MJ Rowan - Molecular brain, 2012 - Springer
Alzheimer's disease (AD) is characterized by episodic memory impairment that often
precedes clinical diagnosis by many years. Probing the mechanisms of such impairment …

The Medical Research Council prion disease rating scale: a new outcome measure for prion disease therapeutic trials developed and validated using systematic …

AGB Thompson, J Lowe, Z Fox, A Lukic, MC Porter… - Brain, 2013 - academic.oup.com
Progress in therapeutics for rare disorders like prion disease is impeded by the lack of
validated outcome measures and a paucity of natural history data derived from prospective …