Prion diseases are fatal brain disorders caused by misfolded prion protein. The best known is Creutzfeldt-Jakob disease (CJD), which occurs at about 1 to 2 deaths per million people per year worldwide. About 85 percent of cases are sporadic, 5 to 15 percent are genetic, and a small number are acquired, as reported by the CDC. The NHS notes there is no cure. These pages state cohort sizes, methods and uncertainty where the sources give them.
Newcomers can begin with the field guide. Then go to diseases, epidemiology, genetics, diagnostics, therapeutics, prevention and biosafety. The atlas holds the underlying entries, search covers the whole site, and partners has briefs for industry and funders. The home page is here.
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- AI in prion researchTwo published machine learning studies in sporadic CJD, one on MRI subtyping and one on survival, with cohort sizes, accuracy and stated limits.
- Transmission and biosafetyPrion biosafety research: infectivity in blood and tissues, decontamination efficacy, animal strain crossover and the containment assays need.
- Diagnostics researchPrion diagnostics research: seeded amplification assays, revised surveillance criteria, the reference laboratories and what remains unvalidated.
- Disease researchResearch hub for the human prion diseases: how they are classified, the sporadic, genetic and acquired groups, and where each has its own literature.
- How common is prion disease?: researchPrion disease epidemiology: national surveillance counts, why recorded sporadic CJD is rising, age-period-cohort evidence and occupational risk analyses.
- Field guide for newcomersA field guide for researchers new to prion disease: the disease types, what past trials taught, current approaches and who works where.
- Genetics researchResearch hub on the genetics of prion disease: PRNP variants, penetrance from population cohorts, codon 129, GWAS loci, transmission ratios and testing.
- MechanismsHub for the biology of prion disease: misfolding, strains, species barriers, neurotoxicity and the function of normal prion protein.
- Open questionsHub for unresolved questions in prion research, from biology and diagnostics to trial endpoints, genetics, zoonotic risk and access to care.
- Prevention researchWhy prevention trials in genetic prion disease are hard to design, what the Neurology 2019 analysis found, and what the PRiSM pre-symptomatic arm registers.
- Research resourcesEntry point to the research resource pages on this site, written for researchers, institutes and laboratories working on prion disease.
- Therapeutics landscapeWhere prion disease therapeutics stand in 2026: PrP-lowering trials, preclinical gene editing, past failed trials and the evidence limits for each.