Prion Diseases (Methods in Molecular Medicine)

Book Info......Page 1
Table of Contents......Page 2
1. Introduction......Page 3
2. Scientific Investigation......Page 4
3. Treatment and Prevention......Page 13
1. Introduction......Page 17
2. Sporadic CJD......Page 19
3. Familial CJD......Page 22
4. latrogenic CJD......Page 25
5. Diagnostic Tests......Page 26
6. Data from the UK National Surveillance Unit......Page 27
7. Conclusions......Page 28
1. Introduction......Page 37
2. Neuropathological Diagnosis......Page 39
3. Relation of Neuropathology to Host Genotype......Page 52
4. Recent Developments in Neuropathology......Page 53
5. Conclusion......Page 54
1. Introduction......Page 61
2. PrPCJD......Page 62
3. Protocols for PrP lmmunocytochemistry......Page 64
4. PrP lmmunostaining in Sporadic CJD Cases......Page 65
5. PrPCJD Staining Patterns in Other Prion Dementias......Page 72
6. Differentiation from Other Dementias......Page 77
7. Role of PrP lmmunocytochemistry in Diagnosis......Page 79
8. Role of PrP lmmunocytochemistry in Furthering the Understanding of Prion Diseases......Page 80
10. Pitfalls in PrPCJD lmmunocytochemistry......Page 81
1. Introduction......Page 87
2. Materials......Page 90
3. Methods......Page 92
1. Introduction......Page 107
2. Accidental Transmission of TDE......Page 108
4. Inactivation Studies on TDE Agents......Page 109
5. Working Practice in the Laboratory......Page 113
2. Case Ascertainment......Page 121
3. Case Definition......Page 128
4. Case-Control Studies......Page 132
5. Conclusions......Page 134
8 - Environmental Causes of Human Spongiform Encephalopathy......Page 141
2. The Identification of BSE in Great Britain......Page 157
3. Case Definition of BSE......Page 158
4. The Initial Epidemiological Studies......Page 159
5. Analytical Epidemiological Studies to Test the Working Hypothesis and to Determine the Occurrence of Other Potential Means of Transmission......Page 165
6. Monitoring the Epidemic......Page 167
7. Further Analytical Studies......Page 169
9. An Epidemiological Epilogue......Page 171
2. Before the Storm......Page 177
3. Monitoring the Epidemic......Page 186
4. The Public Health......Page 189
5. The Role of Research......Page 191
6. Medicinal Products......Page 192
8. CJD Epidemiology......Page 193
9. Relations with the Media......Page 194
11. International Repercussions......Page 195
12. Continued Monitoring......Page 196
13. Conclusion......Page 199
1. Introduction......Page 201
2. Diagnostic Testing......Page 202
3. Presymptomatic Testing......Page 204
4. Prenatal Testing......Page 208
5. Family Management......Page 209
6. Conclusions......Page 210
1. Introduction......Page 213
2. Methods of Genotyping Sheep......Page 216
3. Use of Genotype Information......Page 221
2. Agent Strain Discrimination......Page 225
3. Isolation of Strains of Spongiform Encephalopathies in Mice......Page 229
4. Stability of Mouse-Passaged Strains: Detection of “Mutant” Strains......Page 231
5. Epidemiological Applications of Strain Typing......Page 233
6. The Molecular Basis of Strain Variation......Page 236
1. Introduction......Page 239
2. Methods to Produce PrP-Deficient Mice......Page 240
3. Use of PrP-Deficient Mice in Scrapie Research......Page 243
4. Normal Function of PrP......Page 246
5. Introduction of Point Mutations into the Endogenous Murine PrP Gene by Gene Targeting......Page 247
6. Future Prospects......Page 248
1. Introduction......Page 253
3. PrP Sequences and Species Barriers......Page 254
4. Studies with Syrian Hamster PrP Transgenes......Page 255
5. Studies with Human and Sheep PrP Transgenic Mice......Page 256
6. Enhancing Transmission of Exogenous Prions into TgPrP Mice......Page 257
7. Beyond Primary Structure: Other Components of Species Barriers......Page 258
8. Reprise: General Approaches to Creating Tg(PrP) Mice......Page 259
9. Concluding Remarks......Page 260
1. Introduction......Page 267
2. The Amyloid Protein in GSS Disease......Page 269
3. Assembly and Conformation of PrP Peptides In Vitro......Page 270
4. Biological Effects of PrP Peptides In Vitro......Page 272
5. Methods......Page 275
1. Introduction......Page 287
3. Methods......Page 293
1. Introduction......Page 303
2. Materials and Methods......Page 304
3. Patterns of PrP Accumulation Revealed by lmmunoelectron Microscopy......Page 312