BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

A new variant of Creutzfeldt-Jakob disease in the UK

Will, R. G., Ironside, J. W., Zeidler, M., Estibeiro, K., Cousens, S. N., Smith, P. G., Alperovitch, A., Poser, S., Pocchiari, M., & Hofman, A. (1996) · The Lancet · doi:10.1016/s0140-6736(96)91412-9 · Cited by 2,707

Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent

Bruce, M. E., Will, R. G., Ironside, J. W., McConnell, I., Drummond, D., Suttie, A., McCardle, L., Chree, A., Hope, J., Birkett, C., et al. (1997) · Nature · doi:10.1038/39057 · Cited by 1,967

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

Collinge, J., Sidle, K. C. L., Meads, J., Ironside, J., & Hill, A. F. (1996) · Nature · doi:10.1038/383685a0 · Cited by 1,735

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

Büeler, H., Fischer, M., Lang, Y., Bluethmann, H., Lipp, H.-P., DeArmond, S. J., Prusiner, S. B., Aguet, M., & Weissmann, C. (1992) · Nature · doi:10.1038/356577a0 · Cited by 1,655

The same prion strain causes vCJD and BSE

Hill, A. F., Desbruslais, M., Joiner, S., Sidle, K. C. L., Gowland, I., Collinge, J., Doey, L. J., & Lantos, P. (1997) · Nature · doi:10.1038/38925 · Cited by 1,456

Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease

Palmer, M. S., Dryden, A. J., Hughes, J. T., & Collinge, J. (1991) · Nature · doi:10.1038/352340a0 · Cited by 890

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication

Prusiner, S. B., Scott, M., Foster, D., Pan, K.-M., Groth, D., Mirenda, C., Torchia, M., Yang, S.-L., Serban, D., Carlson, G. A., et al. (1990) · Cell · doi:10.1016/0092-8674(90)90134-z · Cited by 871

Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity

Telling, G. C., Parchi, P., DeArmond, S. J., Cortelli, P., Montagna, P., Gabizon, R., Mastrianni, J., Lugaresi, E., Gambetti, P., & Prusiner, S. B. (1996) · Science · doi:10.1126/science.274.5295.2079 · Cited by 865

Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins

Kascsak, R. J., Rubenstein, R., Merz, P. A., Tonna-DeMasi, M., Fersko, R., Carp, R. I., Wisniewski, H. M., & Diringer, H. (1987) · Journal of Virology · doi:10.1128/jvi.61.12.3688-3693.1987 · Cited by 766

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques

Scott, M., Foster, D., Mirenda, C., Serban, D., Coufal, F., Wälchli, M., Torchia, M., Groth, D., Carlson, G., DeArmond, S. J., et al. (1989) · Cell · doi:10.1016/0092-8674(89)90608-9 · Cited by 697

Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay

Wadsworth, J., Joiner, S., Hill, A., Campbell, T., Desbruslais, M., Luthert, P., & Collinge, J. (2001) · The Lancet · doi:10.1016/s0140-6736(01)05403-4 · Cited by 693

Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy

Bessen, R. A., & Marsh, R. F. (1994) · Journal of Virology · doi:10.1128/jvi.68.12.7859-7868.1994 · Cited by 665

Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease

Collinge, J., Palmer, M. S., & Dryden, A. J. (1991) · The Lancet · doi:10.1016/0140-6736(91)93128-v · Cited by 556

Variant Creutzfeldt-Jakob disease

Collinge, J. (1999) · The Lancet · doi:10.1016/s0140-6736(99)05128-4 · Cited by 512

Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier

Bruce, M., Chree, A., McConnell, I., Foster, J., Pearson, G., & Fraser, H. (1994) · Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences · doi:10.1098/rstb.1994.0036 · Cited by 479

Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent

Bessen, R. A., & Marsh, R. F. (1992) · Journal of Virology · doi:10.1128/jvi.66.4.2096-2101.1992 · Cited by 446

BSE transmission to macaques

Lasmézas, C. I., Deslys, J.-P., Demaimay, R., Adjou, K. T., Lamoury, F., Dormont, D., Robain, O., Ironside, J., & Hauw, J.-J. (1996) · Nature · doi:10.1038/381743a0 · Cited by 433

Linkage of prion protein and scrapie incubation time genes

Carlson, G. A., Kingsbury, D. T., Goodman, P. A., Coleman, S., Marshall, S. T., DeArmond, S., Westaway, D., & Prusiner, S. B. (1986) · Cell · doi:10.1016/0092-8674(86)90875-5 · Cited by 398

Species-barrier-independent prion replication in apparently resistant species

Hill, A. F., Joiner, S., Linehan, J., Desbruslais, M., Lantos, P. L., & Collinge, J. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.97.18.10248 · Cited by 295

Strain-specific prion-protein conformation determined by metal ions

Wadsworth, J. D. F., Hill, A. F., Joiner, S., Jackson, G. S., Clarke, A. R., & Collinge, J. (1999) · Nature Cell Biology · doi:10.1038/9030 · Cited by 295

Scrapie strain variation and mutation

Bruce, M. E. (1993) · British Medical Bulletin · doi:10.1093/oxfordjournals.bmb.a072649 · Cited by 247

Identification of multiple quantitative trait loci linked to prion disease incubation period in mice

Lloyd, S. E., Onwuazor, O. N., Beck, J. A., Mallinson, G., Farrall, M., Targonski, P., Collinge, J., & Fisher, E. M. C. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.101130398 · Cited by 192

Descriptive epidemiology of Creutzfeldt‐Jakob disease in six european countries, 1993–1995

Will, R. G., Alperovitch, A., Poser, S., Pocchiari, M., Hofman, A., Mitrova, E., de Silva, R., D'Alessandro, M., Delasnerie‐Laupretre, N., Zerr, I., et al. (1998) · Annals of Neurology · doi:10.1002/ana.410430611 · Cited by 185

Unaltered susceptibility to BSE in transgenic mice expressing human prion protein

Collinge, J., Palmer, M. S., Sidle, K. C. L., Hill, A. F., Gowland, I., Meads, J., Asante, E., Bradley, R., Doey, L. J., & Lantos, P. L. (1995) · Nature · doi:10.1038/378779a0 · Cited by 181

Unaltered susceptibility to BSE in transgenic mice expressing human prion protein

Collinge, J., Palmer, M. S., Sidle, K. C. L., Hill, A. F., Gowland, I., Meads, J., Asante, E., Bradley, R., Doey, L. J., & Lantos, P. L. (1997) · Nature · doi:10.1038/39116 · Cited by 171

Long-Term Subclinical Carrier State Precedes Scrapie Replication and Adaptation in a Resistant Species: Analogies to Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease in Humans

Race, R., Raines, A., Raymond, G. J., Caughey, B. W., & Chesebro, B. W. (2001) · Journal of Virology · doi:10.1128/jvi.75.21.10106-10112.2001 · Cited by 170

Sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of epidemiological surveillance data for 1970-96

Cousens, S., Zeidler, M., Esmonde, T., Silva, R. D., Wilesmith, J. W., Smith, P. G., & Will, R. G. (1997) · BMJ · doi:10.1136/bmj.315.7105.389 · Cited by 114

Prion protein gene analysis in new variant cases of Creutzfeldt-Jakob disease

Collinge, J., Beck, J. A., Campbell, T., Estibeiro, K., & Will, R. G. (1996) · The Lancet · doi:10.1016/s0140-6736(05)64378-4 · Cited by 114

Aminoacid polymorphism in human prion protein and age at death in inherited prion disease

Baker, H. F., Poulter, M., Crow, T. J., Frith, C., Lofthouse, R., Ridley, R. M., & Collinge, J. (1991) · The Lancet · doi:10.1016/0140-6736(91)92953-y · Cited by 111

Incidence of Creutzfeldt-Jakob disease in Switzerland

Glatzel, M., Rogivue, C., Ghani, A. C., et al. (2002) · The Lancet · doi:10.1016/s0140-6736(02)09384-4 · Cited by 97

Codon 129 genotype and new variant CJD

Zeidler, M., Stewart, G. E., Cousens, S., Estibeiro, K., & Will, R. G. (1997) · The Lancet · doi:10.1016/s0140-6736(05)63366-1 · Cited by 83

IMMUNOSTAINING OF SCRAPIE CEREBRAL AMYLOID PLAQUES WITH ANTISERA RAISED TO SCRAPIE–ASSOCIATED FIBRILS (SAF)

McBride, P., Bruce, M. E., & Fraser, H. (1988) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.1988.tb00892.x · Cited by 61

Florid plaques in ovine PrP transgenic mice infected with an experimental ovine BSE

Crozet, C., Bencsik, A. A., Flamant, F., Lezmi, S., Samarut, J., & Baron, T. (2001) · EMBO Reports · doi:10.1093/embo-reports/kve204 · Cited by 44

Failure to transmit bovine spongiform encephalopathy to marmosets with ruminant-derived meal

Ridley, R. M., Baker, H. F., & Windle, C. P. (1996) · The Lancet · doi:10.1016/s0140-6736(05)64377-2 · Cited by 26

Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice

Ghazi-Noori, S., Froud, K. E., Mizielinska, S., Powell, C., Smidak, M., Fernandez de Marco, M., O’Malley, C., Farmer, M., Parkinson, N., Fisher, E. M. C., et al. (2012) · Brain · doi:10.1093/brain/aws006 · Cited by 115

THE PUBLIC HEALTH IMPACT OF PRION DISEASES

Belay, E. D., & Schonberger, L. B. (2005) · Annual Review of Public Health · doi:10.1146/annurev.publhealth.26.021304.144536 · Cited by 116

A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea

Collinge, J., Whitfield, J., McKintosh, E., Frosh, A., Mead, S., Hill, A. F., Brandner, S., Thomas, D., & Alpers, M. P. (2008) · Philosophical Transactions of the Royal Society B: Biological Sciences · doi:10.1098/rstb.2008.0068 · Cited by 110

Phenotypic variability in human prion diseases

Ironside, J. W., Ritchie, D. L., & Head, M. W. (2005) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2005.00697.x · Cited by 58

Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation

Clayton, E. L., Mancuso, R., Nielsen, T. T., Mizielinska, S., Holmes, H., Powell, N., Norona, F., Overgaard Larsen, J., Milioto, C., Wilson, K. M., et al. (2017) · Human Molecular Genetics · doi:10.1093/hmg/ddx003 · Cited by 32