ArticleActa neuropathologica2017
UK Iatrogenic Creutzfeldt-Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches.
Article in Acta neuropathologica, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 40 citations in OpenAlex.
- Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review.Health technology assessment (Winchester, England) · 2020Pooled it
- Differentiation of Plant and Animal-Derived Cholesterol Using irm-Magnetic resonance in chemistry : MRC · 2026Article
- Limited transmission of V180I genetic Creutzfeldt-Jakob disease in knock-in mice models.The Journal of general virology · 2026Article
- In Vivo Seeding of Amyloid-β Protein and Implications in Modeling Alzheimer's Disease Pathology.Biomolecules · 2025Review
- Biological Fluid Biomarkers in Human Prion Diseases with a Note on Biosafety.Sub-cellular biochemistry · 2025Review
- Enhanced Creutzfeldt-Jakob disease surveillance in the older population: Assessment of a protocol for screening brain tissue donations for prion disease.Brain pathology (Zurich, Switzerland) · 2024Article
- A novel subtype of sporadic Creutzfeldt-Jakob disease with PRNP codon 129MM genotype and PrP plaques.Acta neuropathologica · 2023Article
- Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification.Acta neuropathologica · 2021Article
- Potential for transmission of sporadic Creutzfeldt-Jakob disease through peripheral routes.Laboratory investigation; a journal of technical methods and pathology · 2021Article
- Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients.Acta neuropathologica communications · 2021Article
- Understanding Intra-Species and Inter-Species Prion Conversion and Zoonotic Potential Using Protein Misfolding Cyclic Amplification.Frontiers in aging neuroscience · 2021Review
- Article
- Transmission characteristics of heterozygous cases of Creutzfeldt-Jakob disease with variable abnormal prion protein allotypes.Acta neuropathologica communications · 2020Article
- Characterization of Sporadic Creutzfeldt-Jakob Disease and History of Neurosurgery to Identify Potential Iatrogenic Cases.Emerging infectious diseases · 2020Article
- Core transcriptional regulatory circuits in prion diseases.Molecular brain · 2020Article
- Article
- The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins.Acta neuropathologica communications · 2019Article
- Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans.Viruses · 2019Review
- Recent advances in the histo-molecular pathology of human prion disease.Brain pathology (Zurich, Switzerland) · 2019Review
- Epitope mapping of the protease resistant products of RT-QuIC does not allow the discrimination of sCJD subtypes.PloS one · 2019Article
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
Abstract
Creutzfeldt-Jakob disease (CJD) is the prototypic human prion disease that occurs most commonly in sporadic and genetic forms, but it is also transmissible and can be acquired through medical procedures, resulting in iatrogenic CJD (iCJD). The largest numbers of iCJD cases that have occurred worldwide have resulted from contaminated cadaveric pituitary-derived human growth hormone (hGH) and its use to treat primary and secondary growth hormone deficiency. We report a comprehensive, tissue-based and molecular genetic analysis of the largest series of UK hGH-iCJD cases reported to date, including in vitro kinetic molecular modelling of genotypic factors influencing prion transmission. The results show the interplay of prion strain and host genotype in governing the molecular, pathological and temporal characteristics of the UK hGH-iCJD epidemic and provide insights into the adaptive mechanisms involved when prions cross genotypic barriers. We conclude that all of the available evidence is consistent with the hypothesis that the UK hGH-iCJD epidemic resulted from transmission of the V2 human prion strain, which is associated with the second most common form of sporadic CJD.
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Registered trials
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