Biology

Prion Biology
Research and Advances

Editor: Vincent Béringue, PhD

Prion Biology

Available now.
Pub Date: March 2013
Hardback Price: $149.95 US
Hard ISBN: 9781926895376
Pages: 282pp
Binding Type: hardbound

This title includes a number of Open Access chapters.



This book contains a selection of chapters aimed to provide a better understanding prion structure and biology. Together these chapters provide an overview of prion biology and underscore some of the challenges we face if we want to understand how this lively pathogen propagates and evolves in mammals. There is also mounting evidence that studying prion biology has a wider relevance due to similarities in the processes of protein misfolding and aggregation between prion disorders and other neurodegenerative disorders such as Alzheimer’s, Parkinson’s and Huntington’s diseases.

Mammalian prions are infectious pathogens responsible for transmissible spongiform encephalopathies (TSEs), a group of fatal neurodegenerative disorders affecting farmed and wild animals and humans. Scrapie in sheep and goats, bovine spongiform encephalopathy (BSE), chronic wasting diseases in cervids, and human Creutzfeldt-Jakob disease (CJD) are among the most prevalent TSEs worldwide. Long considered in the middle of the last century as “slow viruses” of the central nervous system, prions now stand firmly as a novel class of proteinaceous infectious pathogens. Prions are essentially composed of PrPSc, a misfolded, aggregation-prone form of the ubiquitously expressed, host-encoded cellular prion protein (PrPC). Due to these unique properties, prions have attracted a strong research interest. Since the “mad cow” crisis, they also have had a profound impact on human and animal health policy and far-reaching implications on the precautionary principle.

CONTENTS:
Introduction
Chapter 1: Prion Protein’s Protection Against Pain
Chapter 2: Prion Protein-Encoding Genes
Chapter 3: Protein Misfolding Cyclic Amplification
Chapter 4: The Amplification of the Scrapie Isoform of Prion Protein
Chapter 5: The Octarepeat Region of the Prion Protein
Chapter 6: Prion Protein Self-Peptides
Chapter 7: PrPSc Spreading Patterns and Prion Types
Chapter 8: Experimental H-Type Bovine Spongiform Encephalopathy
Chapter 9: A Novel Form of Human Disease
Chapter 10: Prions as Seeds and Recombinant Prion Proteins as Substrates
Chapter 11: Quantitative Phosphoproteomic Analysis of Prion-Infected Neuronal Cells
Index


About the Authors / Editors:
Editor: Vincent Béringue, PhD
Molecular Virology Immunology Unit, Institut National de la Recherche Agronomique (INRA, Jouy-en-Josas, France)

Dr. Vincent Béringue is working in the prion field for almost twenty years. He has obtained his PhD in 1998 from the AgroParisTech Institute in Paris, in the laboratory of late Dr. Dominique Dormont. After a MRC-funded postdoctoral position in the laboratory of Prof. John Collinge and Dr. Simon Hawke at Imperial College School of Medicine in London, he joined Dr. Hubert Laude’s laboratory at INRA (National Institute for Agricultural Research) in Jouy-en-Josas in 2001, as permanent staff scientist. He is now head of lab in the Molecular Virology Immunology Department at INRA. His primary research interests include the biochemistry, diversity and evolution of animal and human prions.




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