PRION BIOLOGY 1
1 An Introduction to Prion Biology and Diseases,&S.B. Prusiner 1
2 Development of the Prion Concept,&S.B. Prusiner 89
3 Bioassays of Prions,&S.B. Prusiner, J. Safar, and S.J. DeArmond 143
4 Transmission and Replication of Prions,&S.B. Prusiner, M.R. Scott, S.J. DeArmond, and G. Carlson 187
5 Structural Studies of Prion Proteins, &C. Govaerts, H. Wille, S.B. Prusiner, and F.E. Cohen 243
6 Doppel, a New PrP-like Mammalian Protein,D. Westaway, L.E. Hood, and S.B. Prusiner 283
FUNGAL PRIONS 305
7 Prions of Yeast and Filamentous Fungi: [URF3], [PSI+],[PIN+], and [Het-s],&R.B. Wickner, S. W. Liebman, and S.J. Saupe 305
TRANSGENETICS AND CELL BIOLOGY OF PRIONS 373
8 Knockouts, Knockins, Transgenics, and Transplants in Prion Research,&E. Flechsig, J.C. Manson, R. Barron, A. Aguzzi, and C. Weissmann 373
9 Transgenetic Investigations of the Species Barrier and Prion Strains,&M. Scott, D. Peretz, R.M. Ridley, H.F. Baker, S.J. DeArmond,and S.B. Prusiner 435
10 Cell Biologyof Prions,&D.A. Harris, P.J. Peters, A. Taraboulos, V. Lingappa, S.J. DeArmond,and S.B. Prusiner 483
PRION DISEASES IN ANIMALS 545
11 Scrapie, Chronic Wasting Disease, and Transmissible Mink Encephalopathy,&S.B. Prusiner, E. Williams, J.-L Laplanche, and M. Shinagawa 545
12 Bovine Spongiform Encephalopathy and Related Diseases,&G.A.H. Wells and J. W. Wilesmith 595
HUMAN PRION DISEASES 629
13 Infectious and Sporadic Prion Diseases,&R.G. Will, M.P. Alpers, D. Dormont, and L.B. Schonberger 629
14 Inherited Prion Diseases,&Q. Kong, W.K. Surewicz, R.B. Petersen, W. Zou, S.G. Chen, P. Gambetti,P. Parchi, S. Capellari, L. Goldfarb, P. Montagna, E. Lugaresi, P. Piccardo,and B. Ghetti 673
15 Neuropathologyof Prion Diseases,&S.J. DeArmond, J.W. Ironside, E. Bouzamondo-Bernstein,D. Peretz, and J.R. Fraser 777
METHODOLOGY, BIOSAFETY, AND THERAPEUTICS 857
16 Some Strategies and Methods for the Study of Prions,&S.B. Prusiner, G. Legname, S.J. DeArmond, F.E. Cohen, J. Safar,D. Riesner, and K. Kaneko 857
17 Biosafety Issues in Prion Diseases,&H. Baron, J. Safar, D. Groth, S.J. DeArmond, and S.B. Prusiner 921
18 Therapeutic Approaches to Prion Diseases,&S.B. Prusiner, B.C.H. May, and F.E. Cohen 961
Glossary, 1015
Appendix, 1023
Index, 1033