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The function of the cellular prion protein in health and disease
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 …
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
Introduction Human prion diseases are heterogeneous, and often rapidly progressive,
transmissible neurodegenerative disorders associated with misfolded prion protein (PrP) …
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 …
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 …
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 …
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 …
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 …
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?
Conventional vaccinations and immunotherapies have encountered major roadblocks in
preventing infectious diseases like HIV, influenza, and malaria. These challenges are due to …
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 …
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 …
validated outcome measures and a paucity of natural history data derived from prospective …