Prion protein:
Saved in:
| Other Authors: | , |
|---|---|
| Format: | Book |
| Language: | English |
| Published: |
Cambridge ; San Diego ; Oxford ; London
Elsevier, Academic Press
2017
|
| Edition: | First edition |
| Series: | Progress in molecular biology and translational science
volume 150 |
| Subjects: | |
| Online Access: | Inhaltsverzeichnis |
| Physical Description: | xix, 495 Seiten Illustrationen |
| ISBN: | 9780128112267 |
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MARC
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Record in the Search Index
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| adam_text | Titel: Prion protein
Autor: Legname, Giuseppe
Jahr: 2017
CONTENTS
Contributors xiii
Preface xvii
1. Functions of the Prion Protein 1
Théo Z. Hirsch, Séverine Martin-Lannerée, and Sophie Mouillet-Richard
1. Introduction 2
2. The Basics of PrPc 3
3. Lessons From Knockout Models 5
4. Miscellaneous Roles in CNS Function 6
5. Cell Fate and Differentiation 9
6. A Broad Protective Role Against Stress 12
7. Cell Adhesion and ECM Contacts 14
8. From Partners to Cell Signaling 16
9. Concluding Remarks 20
References 20
2. Copper- and Zinc-Promoted Interdomain Structure in the
Prion Protein: A Mechanism for Autoinhibition of the
Neurotoxic N-Terminus
Eric G.B. Evans and Glenn L. Millhauser
1. Introduction
2. The N-Terminal Toxicity Hypothesis
3. Cu2+ and Zn2+ Promote Interdomain Interaction in eis
4. Disruption of eis Interaction as a Mechanism of Neurotoxicity
5. Concluding Remarks
Acknowledgment
References
3. Cell Biology of Prion Protein
Daniela Sarnataro, Anna Pepe, and Chiara Zurzolo
1. Expression and Function of Prion Protein
2. PrPc: The Principal Actor in Prion Replication
3. Structure and Biosynthesis of PrPc
4. Trafficking of Cellular Prion Protein
5. Several Topological Forms of PrPc
6. Quality Control of Cellular Prion Protein
35
36
40
43
47
51
52
52
57
58
60
62
65
68
70
vi
Contents
7. GPI-Anchor: Structure and Biosynthesis 71
8. Functions of GPI-Anchored Proteins 72
9. GPI-Anchor Function 73
10. GPI-Anchor and Prion 74
References 76
4. Understanding the Effect of Disease-Related Mutations
on Human Prion Protein Structure: Insights From NMR
Spectroscopy 83
Ivana Biljan, Gregor Ile, and Janez Plavec
1. Introduction 84
2. Structural Features of PrPc and PrPSc 85
3. Strategy for Resonance Assignment and NMR Structure Determination
of Human Prion Protein Variants 87
4. Structural Features of Human Prion Proteins With Pathological Mutations 89
5. Structural Features of Human Prion Protein With Protective E219K
Polymorphism 95
6. Concluding Remarks 98
Acknowledgment 98
References 98
5. Structural Modeling of Human Prion Protein s Point Mutations 105
Giulia Rossetti and Paolo Carloni
1. Introduction 106
2. Wild-Type Human Prion Protein 107
3. Prion Protein Variants 111
4. Chimeric Prion Protein 114
5. Conclusions 115
References 115
6. Prion Protein and Genetic Susceptibility to Diseases Caused
by Its Misfolding 123
George A. Carlson
1. Introduction 124
2. Scrapie, Kuru, and Slow Viruses 125
3. Prions and PrP 125
4. Genetic Linkage of Scrapie Incubation Time and the PrP Gene 126
5. Scrapie Strains 127
6. Prnp Knockouts 130
7. PRNP and Familial Prion Diseases 132
Contents
vii
8. PrP Conformation, Prion Strains, and Susceptibility to
Human Prion Diseases 134
9. Genes in Addition to the PrP Gene That Influence Susceptibility
to PrP Prion Diseases 138
10. Concluding Remarks: Genetics and the Prospects for PrP Prion
Disease Therapeutics 139
References 140
7. The Prion Concept and Synthetic Prions 147
Giuseppe Legname and Fabio Moda
1. The Prion Concept 147
2. Natural and Recombinant PrP Can Misfold In Vitro 149
3. Synthetic Prions Are Infectious 150
4. Synthetic Prions Undergo a Process of Adaptation 151
5. Alternative Approaches to Generate Synthetic Prions 152
6. Concluding Remarks 153
References 153
8. Gene Targeted Transgenic Mouse Models in Prion Research 157
Abigail B. Diack, James D. Alibhai, and Jean C. Manson
1. Introduction 158
2. The Mouse as a Model of Prion Disease 158
3. The Influence of Host PrP Expression on Disease Susceptibility 150
4. PrP Mutations and Disease Transmission 160
5. Posttranslational Modifications Can Influence Disease Transmission 162
6. Crossing the Species Barrier and Assessing Zoonotic Potential 163
7. Modeling Human Prion Disease Transmission 166
8. Defining Human Prion Disease Strains and Transmissibility Potential 167
9. Understanding the Function of PrP 170
10. Mechanisms of Neurodegeneration 171
11. Conclusion 173
References 173
9. Transmission and Replication of Prions 181
Alba Marfn-Moreno, Natalia Fernändez-Borges, Juan C. Espinosa,
Olivier Andréoletti, and Juan M. Torres
1. Prion Replication 182
2. Prion Transmission 189
3. Concluding Remarks 196
References 196
viii
Contents
10. Immunology of Prion Protein and Prions 203
Neil A. Mabbott
1. Introduction 204
2. Prions Infect Secondary Lymphoid Organs (SLO) Before the CNS 205
3. The Contrasting Roles of Mononuclear Phagocytes 213
4. B Cells Disseminate Prions Between SLO 216
5. Prion Neuroinvasion From SLO Occurs via the Peripheral
Nervous System 217
6. Microglia Provide a Host-Protective Role in the CNS 218
7. Influence of Aging 220
8. Influence of Inflammation and Pathogen Coinfection 223
9. Prospects for Immunotherapy Against Prions 225
10. Concluding Remarks 226
References 228
11. Phenotypical Variability in Bovine Spongiform Encephalopathy:
Epidemiology, Pathogenesis, and Diagnosis of Classical and
Atypical Forms 241
Cristiano Corona, Elena Vallino Costassa, Barbara lulini, Maria Caramelli,
Elena Bozzetta, Maria Mazza, Rosanna Desiato, Giuseppe Ru,
and Cristina Casalone
1. Introduction 242
2. Epidemiology 243
3. Pathogenesis of Classical and Atypical BSE 247
4. Sampling 248
5. Rapid Tests 250
6. Histopathological Examination 252
7. Immunohistochemistry 254
8. Western Blotting 257
9. Scrapie-Associated Fibrils 258
10. In Vitro Amplification Techniques 259
References 262
12. Scrapie, CWD, and Transmissible Mink Encephalopathy 267
Candace K. Mathiason
1. Scrapie 268
2. Chronic Wasting Disease 271
3. Transmissible Mink Encephalopathy 279
References 281
Contents
ix
13. Infectious and Sporadic Prion Diseases 293
Richard Knight
1. Introduction 294
2. Sporadic OD 295
3. Variably Protease Sensitive Prionopathy 299
4. Iatrogenic OD 300
5. Variant GD 304
References 312
Further Reading 318
14. Neuropathology of Human Prion Diseases 319
Diane L. Ritchie and James W. Ironside
1. Introduction 320
2. Classification of Human Prion Diseases 320
3. The Autopsy in Human Prion Diseases 325
4. Neuropathology 326
5. Methods for the Detection of PrPSc and PrPres in Tissues 336
6. Conclusions 338
Acknowledgments 338
References 338
15. The Structure of the Infectious Prion Protein and Its Propagation 341
Jesus R. Requena and Holger Wille
1. Introduction 342
2. Infectious Prion Protein Structure 343
3. Propagation of infectious Prions 352
4. Future Approaches to Investigate the Structure of Prions 353
5. Concluding Remarks 355
Acknowledgments 355
References 356
16. Protein Misfolding Cyclic Amplification of Infectious Prions 361
Fabio Moda
1. Introduction 361
2. PMCA Applications in Basic Research 363
3. Applications of PMCA as Supportive Test in Prion Diagnosis 365
4. PMCA Applications in Drug Screening 367
5. PMCA Applications to Evaluate Prion Inactivation 368
6. PMCA and Prion-Like Disorders 368
References 369
X
Contents
17. Amplified Detection of Prions and Other Amyloids by RT-QulC
in Diagnostics and the Evaluation of Therapeutics and
Disinfectants 375
Byron Caughey, Christina D. Orru, Bradley R. Groveman,
Andrew G. Hughson, Matteo Manca, Lynne D. Raymond,
Gregory J. Raymond, Brent Race, Eri Saijo, and Allison Kraus
1. Introduction 376
2. Diagnostic Applications of RT-QulC 378
3. Quantitation of Relative Seed Concentrations 381
4. Therapeutic Applications of RT-QulC 382
5. Evaluating Prion Disinfectants Using RT-QulC 383
6. Technical Considerations in RT-QulC Applications 384
7. Adaptation of RT-QulC Platform to Other Protein Folding Diseases 385
Acknowledgments 385
References 386
18. Biochemical Characterization of Prions 389
Michele Fiorini, Matilde Bongianni, Salvatore Monaco,
and Gianluigi Zanusso
1. Introduction 390
2. 1D Analysis of Molecular Strains 393
3. Molecular Analysis of Prion Strains by 2D-PAGE Analysis 396
4. Biochemical Assays to Characterize and Distinguish Prion Strains 401
5. Molecular and Chemicophysical Similarities Between Fluman and Cattle
Transmissible Spongiform Encephalopathies Forms 404
6. Conclusions 405
References 405
Further Reading 407
19. Omics of Prion Diseases 409
Silvia Vanni
1. Introduction 409
2. Genome-Wide Association Studies 410
3. Microarray-Based Trancriptomic Studies 414
4. Sequencing Approach in Prion Disorders 423
5. Epigenomic Studies 424
6. Proteomic Studies 425
7. Concluding Remarks 426
References 427
Contents
xi
20. Therapeutic Approaches to Prion Diseases 433
Annachiara Gandini and Maria Laura Bolognesi
1. Introduction 434
2. Investigational Drugs 435
3. Preclinical Drug Candidates 442
4. Conclusions 448
References 449
21. Biosafety of Prions 455
Edoardo Bistaffa, Martina Rossi, Chiara M.G. De Luca, and Fabio Moda
1. Introduction 456
2. Prion Classification 457
3. Levels of Infectivity in Different Tissues of Animal and Human TSEs 459
4. Risk Assessment 460
5. Infectious Properties of PMCA and RT-QulC Reaction Products 463
6. General Biosafety Recommendations 466
7. Operating Standard Procedures for Working in BSL-2 or BSL-3 Facilities 467
8. Biochemical and Histological Analysis 475
9. Inactivation of Prions and IATA Regulations 476
References 477
Index 487
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| id | DE-604.BV044516263 |
| illustrated | Illustrated |
| indexdate | 2024-07-10T07:54:44Z |
| institution | BVB |
| isbn | 9780128112267 |
| language | English |
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| physical | xix, 495 Seiten Illustrationen |
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| publisher | Elsevier, Academic Press |
| record_format | marc |
| series | Progress in molecular biology and translational science |
| series2 | Progress in molecular biology and translational science |
| spelling | Prion protein edited by Giuseppe Legname (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy), Silvia Vanni (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy) First edition Cambridge ; San Diego ; Oxford ; London Elsevier, Academic Press 2017 xix, 495 Seiten Illustrationen txt rdacontent n rdamedia nc rdacarrier Progress in molecular biology and translational science volume 150 Prionprotein (DE-588)4619314-5 gnd rswk-swf Prion (DE-588)4353535-5 gnd rswk-swf Prion (DE-588)4353535-5 s Prionprotein (DE-588)4619314-5 s DE-604 Legname, Giuseppe (DE-588)103879837X edt Vanni, Silvia edt Progress in molecular biology and translational science volume 150 (DE-604)BV035256576 150 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=029915912&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Prion protein Progress in molecular biology and translational science Prionprotein (DE-588)4619314-5 gnd Prion (DE-588)4353535-5 gnd |
| subject_GND | (DE-588)4619314-5 (DE-588)4353535-5 |
| title | Prion protein |
| title_auth | Prion protein |
| title_exact_search | Prion protein |
| title_full | Prion protein edited by Giuseppe Legname (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy), Silvia Vanni (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy) |
| title_fullStr | Prion protein edited by Giuseppe Legname (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy), Silvia Vanni (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy) |
| title_full_unstemmed | Prion protein edited by Giuseppe Legname (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy), Silvia Vanni (Scuola Internazionale Superiore di Studi Avanzati, SISSA, Trieste, Italy) |
| title_short | Prion protein |
| title_sort | prion protein |
| topic | Prionprotein (DE-588)4619314-5 gnd Prion (DE-588)4353535-5 gnd |
| topic_facet | Prionprotein Prion |
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