Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
Abstract
Strains of transmissible spongiform encephalopathies are distinguished by differing physicochemical properties of PrPSc, the disease-related isoform of prion protein, which can be maintained on transmission to transgenic mice. 'New variant' Creutzfeldt-Jakob disease (CJD) has strain characteristics distinct from other types of CJD and which resemble those of BSE transmitted to mice, domestic cat and macaque, consistent with BSE being the source of this new disease. Strain characteristics revealed here suggest that the prion protein may itself encode disease phenotype.
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The latter kuru case is neuropathologically most similar to type 2 or 3 129 MV type of CJD of the Collinge et al classification or type 2 CJD of the Parchi et al classification.
“The latter case has been neuropathologically compared with known subtypes of CJD and it seems the most similar to type 2 or 3 129 MV type of CJD of the Collinge et al (80) classification or type 2 CJD of the Parchi et al (22) classification.”
From Kuru (2012) · cites this paper as [80]
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- Authors
- Collinge, J., Sidle, K. C. L., Meads, J., Ironside, J., & Hill, A. F.
- Affiliations
- St Mary's Hospital (GB)
- Imperial College London (GB)
- Western General Hospital (GB)
- Venue
- Nature
- Published
- 1996-10
- Volume
- 383
- Issue
- 6602
- Pages
- 685-690
- ISSN
- 0028-0836 (ISSN-L)
- 1476-4687
- OpenAlex
- W2064722614
- Cited by
- 1,735
- References
- 1
- Citations by year
- 2026: 6 · 2025: 15 · 2024: 13 · 2023: 17 · 2022: 31 · 2021: 27 · 2020: 31 · 2019: 21 · 2018: 30 · 2017: 49 · 2016: 40 · 2015: 35 · 2014: 36 · 2013: 59 · 2012: 51
- FWCI
- 35.47
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- Wellcome Trust
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Keywords
- variant Creutzfeldt-Jakob disease, prion disease, PrPSc, prion protein, bovine spongiform encephalopathy, disease phenotypes, molecular epidemiology
- MeSH terms
- Animals, Cats, Glycosylation, Humans, Creutzfeldt-Jakob Syndrome, Macaca, Mice, Inbred C57BL, Mice, Transgenic, Prions, Protein Conformation, Growth Hormone, Genetic Variation, Encephalopathy, Bovine Spongiform, PrPSc Proteins, PrPC Proteins, Endopeptidase K, Mice
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