Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, NIH, Hamilton, MT 59840, USA
Analyses of prion protein deposits in patients with Creutzfeldt-Jakob disease contribute to the controversy over the nature of the transmissible agent (pages 521−525).
REFERENCES
Caughey, B. & Chesebro, B. Prion protein and the transmissible spongiform encephalopathies. Trends Biol. Sci.7, 56−62 (1997). | Article | ChemPort |
Gajdusek, D.C. Transmissible and nontransmissible amyloidoses: Autocatalytic post-translational conversion of host precursor proteins to beta-pleated configurations. J. Neuroimmunol.20, 95−110 (1988). | Article | PubMed | ISI | ChemPort |
Hsiao, K.K. et al. Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science250, 1587−1590 (1990). | PubMed | ISI | ChemPort |
Westaway, D. et al. Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell76, 117−129 (1994). | Article | PubMed | ISI | ChemPort |
Hsiao, K.K. et al. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc. Natl. Acad. Sci. USA91, 9126−9130 (1994). | PubMed | ChemPort |
Brown, P. et al. Human spongiform encephalopathy: The National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann. Neurol.35, 513−529 (1994). | PubMed | ISI | ChemPort |
Silvestrini, M.C. et al. Identification of the prion protein allotypes that accumulate in the brain of sporadic and familial Creutzfeldt-jakob disease patients. Nature Med.3, 521−525 (1997). | Article | PubMed | ISI | ChemPort |
Tagliavini, F. et al. Amyloid fibrils in Gerstmann-Straussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele. Cell79, 695−703 (1994). | PubMed | ISI | ChemPort |
Barbanti, P. et al. Polymorphism at codon 129 or codon 219 of PRNP and clinical heterogeneity in a previously unreported family with Gerstmann-Straussler-Scheinker disease (PrP-P102L mutation). Neurology47, 734−741 (1996). | PubMed | ISI | ChemPort |
Gabizon, R. et al. Insoluble wild-type and protease-resistant mutant protein in brains of patients with inherited prion disease. Nature Med.2, 59−64 (1996). | Article | PubMed | ISI | ChemPort |
Kitamoto, T., Yamaguchi, K., Doh-ura, K. & Tateishi, J.A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Straussler syndrome. Neurology41, 306−310 (1991). | PubMed | ISI | ChemPort |
Goldfarb, L.G. et al. Creutzfeldt-Jacob disease associated with the PRNP codon 200Lys mutation: an analysis of 45 families. Eur. J. Epidemiol.7, 477−486 (1991). | PubMed | ISI | ChemPort |
Pocchiari, M. et al. A new point mutation of the prion protein gene in Creutzfeldt-jakob disease. Ann. Neural.34, 802−807 (1993). | ChemPort |