ArticleProceedings of the National Academy of Sciences of the United States of America2010
Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 125 papers, 1 of them a synthesis that pooled it.
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Who cites it
125 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review.Health technology assessment (Winchester, England) · 2020Pooled it
- The prion protein preference of sporadic Creutzfeldt-Jakob disease subtypes.The Journal of biological chemistry · 2012Trial
- Small protease sensitive oligomers of PrPSc in distinct human prions determine conversion rate of PrP(C).PLoS pathogens · 2012Trial
- A scalable, dividing cell model for the robust propagation and quantification of human sporadic Creutzfeldt-Jakob disease prions.Proceedings of the National Academy of Sciences of the United States of America · 2026Article
- Understanding the Phenotypic Heterogeneity Within the Sporadic Creutzfeldt-Jakob Disease MV1 Subtype.Neuropathology and applied neurobiology · 2026Article
- The Phenotypic Spectrum of Sporadic Creutzfeldt-Jakob Disease Cortical Subtype.Annals of neurology · 2026Article
- Distinct cerebrospinal fluid proteomic signatures define clinicopathological subtypes of sporadic Creutzfeldt-Jakob disease and predict patient survival.Acta neuropathologica communications · 2025Article
- Human PrP E219K: a new and promising substrate for robust RT-QuIC amplification of human prions with potential for strain discrimination.Microbiology spectrum · 2025Article
- Characterization of prion strains and peripheral prion infectivity patterns in E200K genetic CJD patients.Acta neuropathologica · 2025Article
- Transmission and Characterization of Creutzfeldt-Jakob Disease and Chronic Wasting Disease in the North American Deer Mouse.Viruses · 2025Article
- The Evolution of Experimental Rodent Models for Prion Diseases.Journal of neurochemistry · 2025Review
- A Review of CAC-717, a Disinfectant Containing Calcium Hydrogen Carbonate Mesoscopic Crystals.Microorganisms · 2025Review
- Biological Fluid Biomarkers in Human Prion Diseases with a Note on Biosafety.Sub-cellular biochemistry · 2025Review
- Structures of Oligomeric States of Tau Protein, Amyloid-β, α-Synuclein and Prion Protein Implicated in Alzheimer's Disease, Parkinson's Disease and Prionopathies.International journal of molecular sciences · 2024Review
- Unsuccessful transmissions of atypical genetic Creutzfeldt-Jakob disease (PRNP p.T183A-129M) in transgenic mice.Acta neuropathologica · 2024Article
- Characterization of Laboratory-Confirmed Creutzfeldt-Jakob Disease From 3 Ontario Tertiary Care Centers Between 2012 and 2022: A Retrospective Cohort Study.Open forum infectious diseases · 2024Article
- Creutzfeldt-Jakob disease and other prion diseases.Nature reviews. Disease primers · 2024Review
- Sodium hypochlorite inactivation of human CJD prions.PloS one · 2024Article
- "Prion-like" seeding and propagation of oligomeric protein assemblies in neurodegenerative disorders.Frontiers in neuroscience · 2024Review
- Novel histotypes of sporadic Creutzfeldt-Jakob disease linked to 129MV genotype.Acta neuropathologica communications · 2023Article
65 more citing papers are in PubMed but not listed here.
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Authors and funding
3 authors.
Funding
Abstract
The biological determinants of the phenotypic variation in sporadic Creutzfeldt-Jakob disease (sCJD) are unknown. To categorize sCJD cases, the prion protein (PrP) codon 129 genotype and the biochemical characteristics of the disease-associated form of PrP (PrP(Sc)) can be combined to form six subgroups (MM1, MM2, MV1, MV2, VV1, and VV2). This classification largely correlates with the known variation in the clinical and pathological features of sCJD, with the MM1 and MV1 cases representing the "classic" phenotype of sCJD. To address how this classification relates to different strains of sCJD we have inoculated each subgroup of sCJD to a panel of mice expressing different forms of the human PRNP gene (129MM, 129VV, and 129MV). We have established that all subtypes are transmissible to at least one genotype of mouse, and both agent and host factors determine transmission efficiency and the form of PrP(Sc) deposited in the brain. Moreover, we have identified four distinct strains of sCJD using our in vivo strain typing panel.
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