Prion Biology

by Stanley B. Prusiner

Overview

Prions are best known as the agents of mad cow and related diseases, and a growing number of proteins with prion-like properties have been implicated in diseases such as Parkinson's. But some recently discovered prion-like proteins do not appear to induce pathological changes and, in fact, may be key players in basic biological processes such as transcription, immune regulation, and memory formation.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Biology examines the expanding roles of prions and prion-like proteins in health and disease. The contributors review the structures of prions and prion-like proteins, how they aggregate into amyloid fibrils and other insoluble complexes, and the unique biological properties of the aggregated forms (e.g., infectivity, gain/loss of function, and self-templating). The normal physiological roles of prion-like proteins (e.g., CPEB in the brain and MAVS in the immune response) are discussed, as is the formation of abnormal protein aggregates via prion-like mechanisms in various human diseases.

The authors also discuss prions in yeast and other fungi, which are convenient models for studying mechanisms of amyloid formation and propagation. This volume is therefore an essential reference for biochemists, cell and molecular biologists, and all who wish to understand how these alternatively folded, self-propagating proteins function.

Product Details

ISBN-13:	9781621820932
Publisher:	Cold Spring Harbor Laboratory Press
Publication date:	08/31/2017
Series:	Perspectives CSHL
Pages:	456
Product dimensions:	10.20(w) x 7.20(h) x 1.00(d)

Table of Contents

Preface

INTRODUCTION
An Introduction to Prion Biology
Stanley B. Prusiner
The Amyloid Phenomenon and Its Links with Human Disease
Christopher M. Dobson
Toward the Atomic Structure of PrPSc
Jose A. Rodriguez, Lin Jiang, and David S. Eisenberg
The Three-Dimensional Structures of Amyloids
Roland Riek
Protein Quality Control in Health and Disease
Tatyana Dubnikov, Tziona Ben-Gedalya, and Ehud Cohen

FUNGAL PRIONS
Yeast and Fungal Prions
Reed B. Wickner
The HET-S/s Prion Motif in the Control of Programmed Cell Death
Roland Riek and Sven J. Saupe
Prions, Chaperones, and Proteostasis in Yeast
Tatiana A. Chernova, Keith D. Wilkinson, and Yury O. Chernoff

NONPATHOGENIC MAMMALIAN PRIONS
Functional Prions in the Brain
Joseph B. Rayman and Eric R. Kandel
Prion-Like Polymerization in Immunity and Inflammation
Xin Cai, Hui Xu, and Zhijian J. Chen
TIA-1 Is a Functional Prion-Like Protein
Joseph B. Rayman and Eric R. Kandel
Cross-ß Polymerization of Low Complexity Sequence Domains
Masato Kato and Steven L. McKnight

OVERVIEW OF THE PRION DISEASES
Clinical Neurology and Epidemiology of the Major Neurodegenerative Diseases
Michael G. Erkkinen, Mee-Ohk Kim, and Michael D. Geschwind
Molecular Genetics of Neurodegenerative Dementias
Flora I. Hinz and Daniel H. Geschwind
Genetic PrP Prion Diseases
Mee-Ohk Kim, Leonel T. Takada, Katherine Wong, Sven A. Forner, and Michael D. Geschwind
Prion Properties of SOD1 in Amyotrophic Lateral Sclerosis and Potential Therapy
Caroline Sibilla and Anne Bertolotti
Neurodegenerative Disease Transmission and Transgenesis in Mice
Brittany N. Dugger, Daniel P. Perl, and George A. Carlson
Mapping Neurodegenerative Disease Onset and Progression
William W. Seeley
Tau Positron Emission Tomography Imaging
Hartmuth C. Kolb and José Ignacio Andrés
Pathology of Neurodegenerative Diseases
Brittany N. Dugger and Dennis W. Dickson
Potential Pathways of Abnormal Tau and a-Synuclein Dissemination in Sporadic Alzheimer's and Parkinson's Diseases
Heiko Braak and Kelly Del Tredici
Bioassays and Inactivation of Prions
Kurt Giles, Amanda L. Woerman, David B. Berry, and Stanley B. Prusiner
Aggregation and Prion-Like Properties of Misfolded Tumor Suppressors: Is Cancer a Prion Disease?
Danielly C.F. Costa, Guilherme A.P. de Oliveira, Elio A. Cino, Iaci N. Soňares, Luciana P. Rangel, and Jerson L. Silva

Glossary
Index

Editorial Reviews

"Will the articles in this volume be accessible and of interest to those not directly working in prion research or related fields? Again the answer is yes. The editor provides both a very accessible introductory chapter and, importantly, a glossary of the key terms. This will ensure that general readers can navigate the various topics covered in this book and fully appreciate the impact prion research will continue to have on our understanding of major diseases and human and fungal biology." — Quarterly Review of Biology

From the Publisher