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Prions and Diseases / / Wen-Quan Zou and Pierluigi Gambetti, editors
| Prions and Diseases / / Wen-Quan Zou and Pierluigi Gambetti, editors |
| Edizione | [Second edition.] |
| Pubbl/distr/stampa | Cham, Switzerland : , : Springer, , [2023] |
| Descrizione fisica | 1 online resource (773 pages) |
| Disciplina | 573.8639929 |
| Soggetto topico |
Prion diseases
Prions |
| ISBN | 3-031-20565-0 |
| Formato | Materiale a stampa  |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Nota di contenuto |
Intro -- Preface -- Contents -- Part I: History -- Chapter 1: Transmissible Spongiform Encephalopathy: From Its Beginnings to Daniel Carleton Gajdusek -- 1.1 In the Beginning … -- 1.2 Working Out the Biology (in Sheep) -- 1.3 The Mouse That Roared -- 1.4 The Nature of the Beast -- 1.5 The Transition from Biology to Molecular Biology -- 1.6 The Discovery of Kuru -- 1.7 The Kuru-CJD-Scrapie Triangle -- 1.8 Experimental Transmission of Kuru -- 1.9 The Expanding Horizon of Transmissible Spongiform Encephalopathy -- 1.10 Clinical and Epidemiological Precisions -- 1.11 Therapeutic Essays -- 1.12 The End of an Era -- References -- Part II: General Aspects of Prions -- Chapter 2: The Rich Chemistry of the Copper and Zinc Sites in PrPC -- 2.1 Introduction -- 2.2 Brief History -- 2.3 Features of Cu2+ and Zn2+ Coordination in PrP -- 2.4 A Role for Altered Copper Coordination in Octarepeat Expansion Disease -- 2.5 Electrochemical Properties of the PrP Copper Sites -- 2.6 Copper Regulation of PrPC Proteolytic Cleavage -- References -- Chapter 3: PrP Prion Structures -- 3.1 Introduction -- 3.2 Development of Initial Parallel In-Register and 4-Rung β-Solenoid Models for PrPSc Fibrils -- 3.3 Cryo-EM of Synthetic PrP Fibrils -- 3.4 Near-Atomic Cryo-EM Structures of Infectious Tissue-Derived Prions -- 3.5 PrPC to PrPSc Conversion -- 3.6 Impacts of Glycans and GPI Anchors -- 3.7 Structure-Based Modeling of Transmission Barriers -- 3.8 Conclusions -- References -- Chapter 4: Insoluble Cellular Prion Protein and Other Neurodegeneration-Related Protein Aggregates in the Brain of Asymptomatic Individuals -- 4.1 Introduction -- 4.2 Prion Protein Is Characterized by the Presence of an Intrinsically Chameleon-Like Conformation -- 4.3 Insoluble Cellular Prion Protein Aggregates Are Present in Mammalian Brains Without Prion-Infection.
4.4 Spontaneous Formation of the Insoluble Cellular Prion Protein Has Been Modelled with Cultured Cells and May Result from PrP Cytosolic Accumulation -- 4.5 Physiology and Pathophysiology of Insoluble PrPC Aggregates -- 4.5.1 Long-Term Memory Storage -- 4.5.2 Prion Disease -- 4.5.3 Alzheimer's Disease -- 4.6 Insoluble Aβ, Tau, and α-Synuclein Aggregates in the Brain of Asymptomatic Individuals -- 4.7 Conclusions -- References -- Part III: Conversion and Strain of Prions -- Chapter 5: Prion Conversion and Deformed Templating -- 5.1 Introduction -- 5.2 Switching Between Alternative Folding Patterns Within Individual Amyloid Fibrils -- 5.3 Generating Transmissible Prion Diseases De Novo -- 5.4 Experimental Evidence Supporting the Mechanism of Deformed Templating -- 5.5 Deformed Templating In Vivo -- 5.6 Deformed Templating In Vitro -- 5.7 Prion Strain Mutation and Evolution via Deformed Templating -- 5.8 Role of Posttranslational Modifications in Driving Deformed Templating -- 5.9 Deformed Templating as a Mechanism of a Cross-Talk Between Amyloidogenic Proteins -- References -- Chapter 6: Prion Strain Interference -- 6.1 Introduction -- 6.2 Parameters Governing Prion Strain Interference -- 6.2.1 Overview -- 6.2.2 The Interval Between Prion Strains Inoculation Influences Interference -- 6.2.3 The Relative Titer of the Blocking and Superinfecting Strains Can Influence Interference -- 6.2.4 Blocking Strain Replication Is Required for Strain Interference -- 6.2.5 Infection of Common Neuroanatomical Pathways Is Required for Interference -- 6.3 Prion Strain Interference and the Replication Site Hypothesis -- 6.3.1 The Replication Site Hypothesis -- 6.3.2 The Role of PrPC in Prion Strain Interference -- 6.3.3 Prion Strain Interactions and Interconversion -- 6.4 Prion "Vaccination" and Strain Interference -- References. Chapter 7: Molecular Mechanisms Encoding Strains of Prions and Prion-Like Misfolded Proteins -- 7.1 Prion Diversity -- 7.2 Distinct Phenotypes of Prion Strains in Bioassay -- 7.3 Prion Species -- 7.4 Cell Tropism of Prion Strains -- 7.5 Conformational Mechanism of Prion Strain Propagation -- 7.6 Molecular Attributes of Human Prion Strains -- 7.7 Mechanism of Formation, Replication, and Evolution of Human Prions -- 7.8 Expanding the Prion Strain Paradigm to Other Age-Related Neurodegenerative Diseases Caused by Protein Misfolding -- 7.9 Outlook -- References -- Chapter 8: Cofactor Involvement in Prion Propagation -- 8.1 The "Protein-Only" Hypothesis -- 8.2 Components of Purified Native Prions -- 8.3 Prion Replication in Cell-Free Conditions -- 8.4 Formation of Infectious Prions from Minimal Components: Requirement of Non-PrP Cofactor -- 8.5 The Protein X Hypothesis -- 8.6 Non-proteinaceous Prion Cofactors -- 8.7 Potential Roles of Cofactors in Prion Formation and Encoding Infectivity -- 8.8 Additional Roles and Applications for Prion Cofactors -- References -- Chapter 9: Prion Protein Conversion and Lipids -- 9.1 Introduction -- 9.2 Supporting Evidence for the Involvement of Lipids in PrP Conversion -- 9.3 Biophysical Studies of PrP-Lipid Interaction -- 9.4 Analysis of PrP-Lipid Interaction Using Density Gradient and Protease Digestion -- 9.5 The Influence of PrP Mutations on rPrP-Lipid Interaction -- 9.6 Forming Recombinant Prions with Lipid as a Cofactor -- 9.7 Possible Roles of Lipid in Forming an Infectious Prion -- References -- Part IV: Environment and Transmission of Prions -- Chapter 10: Prions in the Environment -- 10.1 Introduction -- 10.2 Prion Sorption to Soil -- 10.3 Prion Transport in the Environment -- 10.4 Degradation and Mitigation of Prions in the Environment -- 10.5 Do Environmental Factors Influence Prion Incidence?. 10.6 Detection of Prions in the Environment -- 10.7 Conclusion -- References -- Chapter 11: Environmentally Acquired Transmissible Spongiform Encephalopathy -- 11.1 Kuru -- 11.2 Creutzfeldt-Jakob Disease -- 11.3 Iatrogenic CJD -- 11.4 The 1970s: Cornea and EEG Depth Electrodes -- 11.5 The 1980s: Human Growth Hormone (hGH) and Dura Mater Grafts -- 11.5.1 Human Growth Hormone -- 11.5.2 Dura Mater -- 11.6 The 1990s: BSE and vCJD -- 11.7 The Millennium: Denouement -- References -- Chapter 12: Risk of Transmission of Creutzfeldt-Jakob Disease by Blood Transfusion -- 12.1 Introduction -- 12.2 Experimental Evidence for Prion Disease Transmission by Blood Transfusion -- 12.2.1 Cellular Prion Protein in Blood -- 12.2.2 Animal Models -- 12.2.3 Rodent Models -- 12.2.4 Primate Models -- 12.2.5 Sheep Models -- 12.3 Evidence for vCJD Transmission by Blood Transfusion and Plasma -- 12.3.1 Secondary Transmission of vCJD by Blood Transfusion -- 12.3.2 Evidence for vCJD Transmission by Plasma Products -- 12.4 Evidence for sCJD Transmission by Blood Transfusion and Plasma -- 12.5 Methods to Detect Prions in Blood and the Prospect of Implementation of a Blood Screening Test for vCJD -- 12.5.1 The Challenge -- 12.5.2 Approaches to Sensitive Detection of PrPSc -- 12.5.3 PrPSc Amplification and Current Blood Test Development -- 12.5.4 Future Perspectives -- 12.6 Conclusion -- References -- Chapter 13: Species Barriers in Prion Disease -- 13.1 Introduction -- 13.2 Prion Protein and Prion Species Barriers -- 13.3 Role of PrP Amino Acid Sequence -- 13.3.1 Region of PrP Involved in Rodent Species Barriers -- 13.3.2 Influence of Single Amino Acid Residues -- 13.3.3 Effect of Prnp Heterozygosity -- 13.4 Influence of PrP Post-translational Modifications -- 13.4.1 Glycosylation -- 13.4.2 GPI Anchor -- 13.5 Non-PrP Host Factors. 13.6 Prion Protein Structure and Prion Species Barriers -- 13.6.1 Structural Regions of PrPC Implicated in Species Barriers -- 13.6.2 Effect of Variable PrPSc Conformation -- 13.7 Molecular Model of Prion Species Barriers -- 13.7.1 Initial Prion Infection and Species Barriers -- 13.7.2 Prion Adaptation and Species Barriers -- 13.8 Intermediate Species and Prion Species Barriers -- 13.8.1 Altered Properties of BSE After Passage into New Species -- 13.8.2 CWD Host Range and Species Barriers -- References -- Part V: Modelling of Prions -- Chapter 14: Modeling the Cell Biology of Prions -- 14.1 Cellular Cultures Supporting TSE Agent Replication -- 14.2 Cell Models of Pathogenic Mutations in the Prion Protein -- 14.3 Conclusion -- References -- Chapter 15: Transgenic Mice Modelling in Prion Diseases -- 15.1 Introduction -- 15.2 Host PrP and Susceptibility to TSEs -- 15.3 Transmission of Agent Within a Host -- 15.4 Transport into the LRS -- 15.5 Crossing the Species Barrier and Strain Adaptation -- 15.6 Defining Strains of TSE Agents -- 15.7 Mechanisms of Neurodegeneration -- 15.8 Conclusion -- References -- Chapter 16: Stem Cell Models in Prion Research -- 16.1 Introduction -- 16.2 Stem Cell Models Used in Prion Research -- 16.3 Stem Cell Models for Elucidating Prion Cell Biology and Function -- 16.4 Stem Cell Models for Understanding Normal PrP Function in Progenitors -- 16.5 Murine Stem Cell Models for Studying Prion Disease -- 16.6 Human Stem Cell Models of Prion Disease -- 16.6.1 Infectious Disease -- 16.6.2 Genetic Disease -- 16.7 Other Stem Cell Models -- 16.8 Summary -- References -- Chapter 17: Drosophila Models of Prion Diseases -- 17.1 The Prion Protein in Disease -- 17.2 Investigating Biological Processes in Animal Models -- 17.3 Modeling Prion Diseases in Drosophila -- 17.3.1 Modeling Neurodegeneration in Flies. 17.3.2 Early Fly Models of Prion Disease. |
| Record Nr. | UNINA-9910637724203321 |
| Cham, Switzerland : , : Springer, , [2023] | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
| ||
Prions and Diseases : Volume 1, Physiology and Pathophysiology / / edited by Wen-Quan Zou, Pierluigi Gambetti
| Prions and Diseases : Volume 1, Physiology and Pathophysiology / / edited by Wen-Quan Zou, Pierluigi Gambetti |
| Edizione | [1st ed. 2013.] |
| Pubbl/distr/stampa | New York, NY : , : Springer New York : , : Imprint : Springer, , 2013 |
| Descrizione fisica | 1 online resource (244 p.) |
| Disciplina | 579.29 |
| Soggetto topico |
Neurosciences
Human physiology Neurology Proteins Animal physiology Biochemistry Human Physiology Neurology Protein Science Animal Physiology Biochemistry, general |
| ISBN |
1-283-91173-6
1-4614-5305-4 |
| Formato | Materiale a stampa |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Nota di contenuto | Transmissible Spongiform Encephalopathy: from the High Middle Ages to Daniel Carlton Gajdusek -- The Rich Chemistry of the Copper and Zinc Sites in Cellular Prion Protein -- Role of Cellular Prion Protein in the Amyloid-ß Oligomer Pathophysiology of Alzheimer’s Disease -- Cellular Prion Protein and Cancers -- Insoluble Cellular Prion Protein -- Protein Misfolding Cyclic Amplification -- Cofactor Involvement in Prion Propagation -- Prion Protein Conversion and Lipids -- New Perspectives on Prion Conversion: Introducing a Mechanism of Deformed Templating -- Infectious and Pathogenic Forms of Prion Protein -- Cellular Mechanisms of Propagation and Clearance -- Molecular Mechanisms Encoding Quantitative and Qualitative Traits of Prion Strains -- Modeling the Cell Biology of Prions -- Prion Strain Interference -- Introduction to Yeast and Fungal Prions -- Yeast Prions are Pathogenic, in-register Parallel Amyloids. |
| Record Nr. | UNINA-9910437839403321 |
| New York, NY : , : Springer New York : , : Imprint : Springer, , 2013 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
| ||
Prions and diseases . Volume 2 Animals, humans and the environment / / Wen-Quan Zou, Pierluigi Gambetti, editors
| Prions and diseases . Volume 2 Animals, humans and the environment / / Wen-Quan Zou, Pierluigi Gambetti, editors |
| Pubbl/distr/stampa | New York, : Springer, 2013 |
| Descrizione fisica | 1 online resource (296 p.) |
| Disciplina | 616.83 |
| Altri autori (Persone) |
ZouWen-Quan
GambettiPierluigi |
| Soggetto topico |
Prion diseases
Prions |
| ISBN |
1-283-91187-6
1-4614-5338-0 |
| Formato | Materiale a stampa |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Nota di contenuto | Bovine Spongiform Encephalopathy -- Classical and Atypical Scrapie in Sheep and Goats -- Sporadic Human Prion Disease -- Environmentally-acquired Transmissible Spongiform -- Prions in the Environment -- The Spectrum of Tau Pathology in Human Prion Disease -- Risk of Transmission of Creutzfeldt-Jakob Disease by Blood Transfusion -- Species Barriers in Prion Disease -- Transgenic Mice Modelling -- Prion Transmission Studies in Transgenic Mice -- Alternative Models of Prion Diseases -- Diagnosis of Prion Disease: Conventional Approaches -- Quaking-induced Conversion (QuIC) Assays for the Detection and Diagnosis of Prion Diseases -- Overview on Treatment of Prion Diseases and Decontamination of Prions -- Immunomodulation. |
| Record Nr. | UNINA-9910437824903321 |
| New York, : Springer, 2013 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
| ||