PubMed · 9767039
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L Manuelidis. 1998-09-25. Cleaning CJD-contaminated instruments.. https://doi.org/10.1126/science.281.5385.1961c
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A recent series of papers has raised issues regarding estimation of the key epidemiological parameters of variant Creutzfeldt-Jakob disease (vCJD) from fitting survival models to case data. In particular, it was stated that the scale of the epidemic cannot be estimated and must be fixed in any analysis. We show that this problem is an artefact of the approximate likelihood used in these papers to facilitate model-fitting, and is not a concern if estimation is based on the full likelihood. We also address comments in these papers regarding the choice of incubation period distribution.
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Inherited prion diseases are neurodegenerative disorders caused by autosomal dominant mutations in the human prion protein gene (PRNP). Kindred with inherited prion disease can show remarkable phenotypic variability that has yet to be explained. Here we report analysis of protease resistant disease-related prion protein (PrP(Sc)) isoforms from a range of inherited prion disease cases (point mutations P102L, D178N, E200K and 2-, 4- and 6-octapeptide repeat insertions) and show that the glycoform ratios of PrP(Sc) associated with PRNP point mutations are distinct from those observed in sporadic, iatrogenic and variant Creutzfeldt-Jakob disease. Patients with the same PRNP mutation can also propagate PrP(Sc) with distinct conformations. These data extend the spectrum of recognized PrP(Sc) types seen in human prion diseases and provide further insight into the generation of diverse clinicopathological phenotypes associated with inherited prion disease.