Prion Diseases

Known as: Prion-Associated Disorders, Transmissible Dementia, Prion Disease 
A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases… (More)
National Institutes of Health

Papers overview

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Review
2017
Review
2017
INTRODUCTION A classification framework for posterior cortical atrophy (PCA) is proposed to improve the uniformity of definition… (More)
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Review
2007
Review
2007
Prions are lethal mammalian pathogens composed of aggregated conformational isomers of a host-encoded glycoprotein and which… (More)
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Highly Cited
2005
Highly Cited
2005
Neurodegenerative diseases such as Alzheimer's, Parkinson's and the transmissible spongiform encephalopathies (TSEs) are… (More)
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Review
2004
Review
2004
Neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), amyotrophic… (More)
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Review
2001
Review
2001
Prion diseases are transmissible neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine… (More)
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Highly Cited
1999
Highly Cited
1999
BACKGROUND Prion diseases are associated with the accumulation of an abnormal isoform of cellular prion protein (PrPSc), which is… (More)
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Highly Cited
1997
Highly Cited
1997
Epidemiological and clinicopathological studies, allied with pathological prion protein (PrPSc) analysis, strongly support the… (More)
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Review
1997
Review
1997
Bovine spongiform encephalopathy (BSE) and human Creutzfeldt-Jakob disease (CJD) are among the most notable central nervous… (More)
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Highly Cited
1996
Highly Cited
1996
The 'protein only' hypothesis postulates that the prion, the agent causing transmissible spongiform encephalopathies, is PrP(Sc… (More)
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Review
1991
Review
1991
Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy of… (More)
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