Prions : current progress in advanced research / edited by Akikazu Sakudo and Takashi Onodera.

Format:

Book

Contributor:

Sakudo, Akikazu, editor.

Onodera, Takashi, 1947- editor.

Language:

English

Subjects (All):

Prion diseases.

Prions.

Physical Description:

1 online resource (162 pages)

Edition:

Second edition.

Place of Publication:

Norfolk, UK : Caister Academic Press, [2019]

Summary:

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.

Contents:

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

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

Idiopathic human prion disease

Genetic prion disease

Acquired human prion disease

7. Inactivation Methods for Prions

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.

Notes:

Description based on print version record.

Description based on publisher supplied metadata and other sources.

ISBN:

1-910190-96-9

OCLC:

1090380425