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Prion disease incubation

There is widespread concern that a major epidemic of variant Creautzfeldt-Jakob disease (vCJD) will arise as a result of infection with the bovine spongiform encephalopathy (BSE) prion strain. Polymorphisms of the prion protein gene affect disease susceptibility and incubation periods. In the May 22 Proceedings of the National Academy of Sciences, Lloyd et al. describe a search for quantitative trait loci (QTLs) that affect prion disease incubation times in different mouse strains (Proc Natl Acad Sci USA 2001, 98:6279-6283). They examined 1009 animals from F2 intercrosses between two mouse strains that differ in disease pathogenesis following intracerebral inoculation with scrapie prions, CAST/Ei (incubation period of 188 days) and NZW/OlaHsd (incubation period of 108 days). Lloyd et al.found evidence for linkage with eight QTLs on three chromosomes (mouse chromosomes 2, 11 and 12). On the basis of these results, they caution against the validity of epidemiological predictions of vCJD that are based on genetic models of current patients who have shown short incubation periods.

References

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    Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD.

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    Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene.

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    Proceedings of the National Academy of Sciences, [http://www.pnas.org]

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Keywords

  • Quantitative Trait Locus
  • Incubation Period
  • Mouse Strain
  • Prion Protein
  • Prion Disease