The Prion Protein | Book

Publisher: Savanna Press
Edited by: Jörg Tatzelt
Publication date: January 2010
ISBN: 978-0-9543335-2-2
Price: GB £60 or US $99 or EUR69 (paperback).
Pages: viii + 78

A conformational transition of the cellular prion protein (PrPC) into an aberrantly folded isoform designated scrapie prion protein (PrPSc) is the hallmark of a variety of neurodegenerative disorders collectively called prion diseases. They include Creutzfeldt-Jakob disease and Gerstmann-Stäussler-Scheinker syndrome in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and chronic wasting disease (CWD) in free-ranging deer. In contrast to the deadly properties of misfolded PrP, PrPC seems to possess a neuroprotective activity. More-over, animal models indicated that the stress-protective activity of PrPC and the neurotoxic effects of PrPSc are somehow interconnected.

In this timely book, leading scientists in the field have come together to highlight the apparently incongruous activities of different PrP conformers. The articles outline current research on celluar pathways implicated in the formation and signaling of neurotoxic and physiological PrP isoforms and delineate future research direction. Topics covered include the physiologcial activity of PrPC and its possible role as a neurotrophic factor, the finding that aberrant PrP conformers can cause neurodegeneration in the absence of infectious prion propagation, the requirement of the GPI anchor of PrPC for the neurotoxic effects of scrapie prions, the pathways implicated in the formation and neurotoxic properties of cytosolically localized PrP, the impact of metal ions on the processing of PrP, and the role of autophagy in the propagation and clearance of PrPSc. The book is fully illustrated and chapters include comprehensive reference sections.

Essential reading for scientists involved in prion research.

The Prion Protein: Friend and Foe
Jörg Tatzelt

Chapter 1
Prion Neurotoxicity: Insights from Prion Protein Mutants
Isaac H. Solomon, Jessie A. Schepker and David A. Harris

Chapter 2
Prion Protein: Orchestrating Neurotrophic Activities
Vilma R. Martins, Flavio H. Beraldo, Glaucia N. Hajj, Marilene H. Lopes, Kil Sun Lee, Marco M. Prado and Rafael Linden

Chapter 3
Autophagy, Prion Infection and their Mutual Interactions
Andreas Heiseke, Yasmine Aguib and Hermann M. Schatzl

Chapter 4
Prion Protein and Metal Interaction: Physiological and Pathological Implications
Neena Singh, Dola Das, Ajay Singh and Maradumane L. Mohan

Chapter 5
Targeting of the Prion Protein to the Cytosol: Mechanisms and Consequences
Margit Miesbauer, Angelika S. Rambold, Konstanze F. Winklhofer and Jörg Tatzelt

Chapter 6
The Role of GPI-anchored PrPC in Mediating the Neurotoxic Effect of Scrapie Prions in Neurons
Helois E. Radford and Giovanna R. Mallucci

The chapters in this book were originally published as articles in Current Issues in Molecular Biology and are reprinted with permission of the publishers.