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

Related topics

Related topics

25 relations
Central Nervous SystemCommunicable DiseasesCorticostriatal-Spinal DegenerationDementia
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Narrower (4)

Creutzfeldt-Jakob diseaseGerstmann-Straussler-Scheinker DiseaseKuruSPONGIFORM ENCEPHALOPATHY WITH NEUROPSYCHIATRIC FEATURES

Broader (1)

Organic brain syndrome

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
2017
Review
2017
Consensus classification of posterior cortical atrophy
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
A general model of prion strains and their pathogenicity.
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
The most infectious prion protein particles
Neurodegenerative diseases such as Alzheimer's, Parkinson's and the transmissible spongiform encephalopathies (TSEs) are… (More)
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Review
2004
Review
2004
Protein aggregation and neurodegenerative disease
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 of humans and animals: their causes and molecular basis.
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
Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples
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
The same prion strain causes vCJD and BSE
Epidemiological and clinicopathological studies, allied with pathological prion protein (PrPSc) analysis, strongly support the… (More)
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Review
1997
Review
1997
Prion diseases and the BSE crisis.
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
Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.
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
Molecular biology of prion diseases.
Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy of… (More)
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