Prions : Current Progress in Advanced Research.

Intro -- Contents -- Current Books of Interest -- Preface -- 1. Introduction to Current Progress in Advanced Research on Prions -- Risk assessment in food safety and for blood products -- Protein misfolding neurodegenerative diseases -- Prion clearance -- 2. Function of Prion Protein and the Family Member, Shadoo -- PrPC protects neurons from stress-induced apoptosis -- PrPC mediates toxic signalling by PrPSc -- Stress-inducible and toxic signalling mediated by PrPC are interconnected -- Shadoo, a highly conserved glycoprotein with similarities to PrPC -- Sho is stress-protective, however does not mediate PrPSc-induced toxicity -- Sho mutants devoid of the internal hydrophobic domain do not acquire a toxic potential -- Ablation of PrP in higher organism -- 3. Effect of Microglial Inflammation in Prion Disease -- Background -- Microglial inflammation in the central nervous system -- CXCR3 accelerates prion replication but prolongs survival times after prion infection -- Neurotoxic prion peptide induces IL-1β production in microglia via inflammasome -- Antioxidant cellular prion protein might contribute to control inflammasome in microglia -- Conclusion -- 4. Prion Propagation, its Neurotoxicity and Protein Trafficking -- Introduction -- Prion proteins -- Prions propagate through a positive-feedback mechanism -- Prions impairs post-Golgi vesicular trafficking -- Post-Golgi trafficking disturbance is an early pathogenic event in prion diseases -- Mechanisms of the disturbed trafficking of membrane proteins in prion diseases -- Implications in the pathogenesis of prion diseases -- Conclusions -- 5. Molecular Mechanisms of Prion Diseases -- Introduction -- Prion diseases in humans and animals -- The prion protein -- The gene encoding the prion protein -- Misfolding of the prion protein -- Mechanisms of neuronal death in prion diseases.

Shedding and anchorless PrP -- Prions and the brain -- Prion diseases and non-central nervous system organs -- Outlook -- 6. Clinical Aspects of Human Prion Diseases -- Introduction -- Idiopathic human prion disease -- Genetic prion disease -- Acquired human prion disease -- 7. Inactivation Methods for Prions -- Introduction -- High-risk procedures and patients -- Inactivation methods of prions -- Future perspectives -- 8. Bovine Spongiform Encephalopathy and Scrapie -- Bovine spongiform encephalopathy -- Atypical BSE -- Scrapie -- Atypical scrapie -- 9. Chronic Wasting Disease: Current Assessment of Transmissibility -- History of chronic wasting disease (CWD) and its current status -- Susceptibility and environmental reservoirs of CWD -- Clinical symptoms and pathogenesis of CWD -- CWD strains -- Relationship between CWD and human prion diseases -- Diagnostic methods for CWD -- Conclusion and perspectives -- 10. Future Perspectives in Prion Research -- Breeding with resistant rams -- PMCA, RT-QuIC, and prion-associated activities -- Prion and other neurodegenerative diseases -- Prions and environment contamination -- CWD infection in primates -- Index.

The book opens with an introductory chapter that provides an overview. This is followed by four chapters (chapters 2-5) dealing with fundamental aspects of prion biology, including functions of the cellular isoform of prion protein (PrPC) and molecular mechanisms of prion diseases. The next two chapters (chapters 6-7) focus on clinical aspects of human prion diseases and current approaches for effective inactivation methods. The last part of the book (chapters 8-9) summarizes animal prion diseases, including BSE, scrapie and chronic wasting disease (CWD). In the final chapter, Professor Onodera discusses the likely future direction of research. This book is essential reading for everyone working with prions from the PhD student to the experienced scientist, in academia, the pharmaceutical or biotechnology industries and for those working in clinical environments.

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Electronic reproduction. Ann Arbor, Michigan : ProQuest Ebook Central, 2024. Available via World Wide Web. Access may be limited to ProQuest Ebook Central affiliated libraries.