Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans
- 21 December 1999
- journal article
- Published by Proceedings of the National Academy of Sciences in Proceedings of the National Academy of Sciences
- Vol. 96 (26) , 15137-15142
- https://doi.org/10.1073/pnas.96.26.15137
Abstract
There is growing concern that bovine spongiform encephalopathy (BSE) may have passed from cattle to humans. We report here that transgenic (Tg) mice expressing bovine (Bo) prion protein (PrP) serially propagate BSE prions and that there is no species barrier for transmission from cattle to Tg(BoPrP) mice. These same mice were also highly susceptible to a new variant of Creutzfeldt–Jakob disease (nvCJD) and natural sheep scrapie. The incubation times (≈250 days), neuropathology, and disease-causing PrP isoforms in Tg(BoPrP)Prnp 0/0 mice inoculated with nvCJD and BSE brain extracts were indistinguishable and differed dramatically from those seen in these mice injected with natural scrapie prions. Our findings provide the most compelling evidence to date that prions from cattle with BSE have infected humans and caused fatal neurodegeneration.Keywords
This publication has 49 references indexed in Scilit:
- Is scrapie solely a genetic disease?Nature, 1997
- Association between natural scrapie and PrP genotype in a flock of Suffolk sheep in ScotlandVeterinary Record, 1997
- Transmission of the BSE Agent to Mice in the Absence of Detectable Abnormal Prion ProteinScience, 1997
- Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion DiversityScience, 1996
- Transmission dynamics and epidemiology of BSE in British cattleNature, 1996
- BSE transmission to macaquesNature, 1996
- Prediction of future BSE spreadNature, 1996
- A new variant of Creutzfeldt-Jakob disease in the UKPublished by Elsevier ,1996
- Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agentNeuron, 1995
- Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie.Genes & Development, 1994