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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… 
National Institutes of Health

Related topics

Related topics

25 relations
Central Nervous SystemCommunicable DiseasesCorticostriatal-Spinal DegenerationDementia

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
2008
Review
2008
The prion's elusive reason for being.
The protein-only hypothesis posits that the infectious agent causing transmissible spongiform encephalopathies consists of… 
Highly Cited
2008
Highly Cited
2008
A novel human disease with abnormal prion protein sensitive to protease
To report a novel prion disease characterized by distinct histopathological and immunostaining features, and associated with an… 
Review
2004
Review
2004
Copper binding in the prion protein.
A conformational change of the prion protein is responsible for a class of neurodegenerative diseases called the transmissible… 
Review
2004
Review
2004
Emerging principles of conformation-based prion inheritance.
The prion hypothesis proposes that proteins can act as infectious agents. Originally formulated to explain transmissible… 
Highly Cited
2003
Highly Cited
2003
Balancing Selection at the Prion Protein Gene Consistent with Prehistoric Kurulike Epidemics
Kuru is an acquired prion disease largely restricted to the Fore linguistic group of the Papua New Guinea Highlands, which was… 
Review
2002
Review
2002
Neuroinflammation in Alzheimer's disease and prion disease
Alzheimer's disease (AD) and prion disease are characterized neuropathologically by extracellular deposits of Aβ and PrP amyloid… 
Highly Cited
2002
Highly Cited
2002
Post‐natal knockout of prion protein alters hippocampal CA1 properties, but does not result in neurodegeneration
Prion protein (PrP) plays a crucial role in prion disease, but its physiological function remains unclear Mice with gene… 
Highly Cited
2000
Highly Cited
2000
NMR solution structure of the human prion protein.
The NMR structures of the recombinant human prion protein, hPrP(23-230), and two C-terminal fragments, hPrP(90-230) and hPrP(121… 
Highly Cited
1995
Highly Cited
1995
Sulfated Glycans Stimulate Endocytosis of the Cellular Isoform of the Prion Protein, PrPC, in Cultured Cells (*)
There is currently no effective therapy for human prion diseases. However, several polyanionic glycans, including pentosan… 
Highly Cited
1994
Highly Cited
1994
Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism.
Fatal familial insomnia and a subtype of Creutzfeldt-Jakob disease, two clinically and pathologically distinct diseases, are… 
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