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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.
Highly Cited
2005
Highly Cited
2005
Elimination of transmissible spongiform encephalopathy infectivity and decontamination of surgical instruments by using radio-frequency gas-plasma treatment.
It has now been established that transmissible spongiform encephalopathy (TSE) infectivity, which is highly resistant to… 
Highly Cited
2004
Highly Cited
2004
Antigenic characterization of an abnormal isoform of prion protein using a new diverse panel of monoclonal antibodies.
Highly Cited
2002
Highly Cited
2002
Antagonistic Interactions between Yeast [PSI+] and [URE3] Prions and Curing of [URE3] by Hsp70 Protein Chaperone Ssa1p but Not by Ssa2p
ABSTRACT The yeast [PSI +], [URE3], and [PIN +] genetic elements are prion forms of Sup35p, Ure2p, and Rnq1p, respectively… 
Highly Cited
2002
Highly Cited
2002
Cellular Phenotyping of Secretory and Nuclear Prion Proteins Associated with Inherited Prion Diseases*
The pathogenic mechanisms leading from mutations in the prion protein (PrP) gene to infectious disease are not understood. To… 
Review
2001
Review
2001
Three-dimensional structures of prion proteins.
Highly Cited
2001
Highly Cited
2001
Changing a single amino acid in the N‐terminus of murine PrP alters TSE incubation time across three species barriers
The PrP gene of the host exerts a major influence over the outcome of transmissible spongiform encephalopathy (TSE) disease, but… 
Highly Cited
2000
Highly Cited
2000
Brain Copper Content and Cuproenzyme Activity Do Not Vary with Prion Protein Expression Level*
Prion diseases are neurodegenerative disorders that result from conformational transformation of a normal cell surface… 
Review
1996
Review
1996
For Protein Misassembly, It's the “I” Decade
Highly Cited
1996
Highly Cited
1996
Strain specific and common pathogenic events in murine models of scrapie and bovine spongiform encephalopathy.
The development of transmissible spongiform encephalopathies in experimental models depends on two major factors: the… 
Highly Cited
1987
Highly Cited
1987
Purified prion proteins and scrapie infectivity copartition into liposomes.
Considerable evidence indicates that the scrapie prion protein (PrP 27-30) is required for infectivity. Aggregates of PrP 27-30… 
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