Contents

Preface

INTRODUCTION

An Introduction to Prion Diseases

Stanley B. Prusiner

PrP

Prion Strain Diversity

Jason C. Bartz

Biology and Genetics of PrP Prion Strains

Sina Ghaemmaghami

Experimental Models of Inherited PrP Prion Diseases

Joel C. Watts and Stanley B. Prusiner

Sporadic and Infectious Human Prion Diseases

Robert G. Will and James W. Ironside

Molecular Mechanisms of Chronic Wasting Disease Prion Propagation

Julie A. Moreno and Glenn C. Telling

Structural Biology of PrP Prions

Gerald Stubbs and Jan Stöhr

TAU

Cellular Models for the Study of Prions

Brandon B. Holmes and Marc I. Diamond

The Prion-Like Behavior of Assembled Tau in Transgenic Mice

Florence Clavaguera, Markus Tolnay, and Michel Goedert

Interactions between Microtubule-Associated Protein Tau (MAPT) and Small Molecules

Jennifer N. Rauch, Steven H. Olson, and Jason E. Gestwicki

Chronic Traumatic Encephalopathy: Is Latency in Symptom Onset Explained by Tau Propagation

Joshua Kriegel, Zachary Papadopoulos, and Ann C. McKee

Aβ

The Prion-Like Properties of Amyloid-β Assemblies: Implications for Alzheimer's Disease

Lary C. Walker, Juliane Schelle, and Mathias Jucker

β-Amyloid Prions and the Pathobiology of Alzheimer's Disease

Joel C. Watts and Stanley B. Prusiner

Genetics of β-Amyloid Precursor Protein in Alzheimer's Disease

Julia TCW and Alison M. Goate

Structural and Chemical Biology of Presenilin Complexes

Douglas S. Johnson, Yue-Ming Li, Martin Pettersson, and Peter H. St George-Hyslop

Binding Sites for Amyloid-β Oligomers and Synaptic Toxicity

Levi M. Smith and Stephen M. Strittmatter

Molecular Structure of Aggregated Amyloid-β: Insights from Solid-State Nuclear Magnetic Resonance

Robert Tycko

α-SYNUCLEIN

Cell Biology and Pathophysiology of α-Synuclein

Jacqueline Burre, Manu Sharma, and Thomas C. Südhof

α-Synuclein: Experimental Pathology

Masato Hasegawa, Takashi Nonaka, and Masami Masuda-Suzukake

α-Synuclein: Multiple System Atrophy Prions

Amanda L. Woerman, Joel C. Watts, Atsushi Aoyagi, Kurt Giles, Lefkos T. Middleton, and Stanley B. Prusiner

The Transcellular Propagation and Intracellular Trafficking of α-Synuclein

George K. Tofaris, Michel Goedert, and Maria Grazia Spillantini

Genetics of Synucleinopathies

Robert L. Nussbaum

TDP-43, FUS, and C9ORF72

Clinical Spectrum of Amyotrophic Lateral Sclerosis (ALS)

Leslie I. Grad, Guy A. Rouleau, John Ravits, and Neil R. Cashman

Genetics of Amyotrophic Lateral Sclerosis

Mehdi Ghasemi and Robert H. Brown, Jr.

Biological Spectrum of Amyotrophic Lateral Sclerosis Prions

Magdalini Polymenidou and Don W. Cleveland

Biology and Pathobiology of TDP-43 and Emergent Therapeutic Strategies

Lin Guo and James Shorter

TDP-43 Prions

Takashi Nonaka and Masato Hasegawa

Fused in Sarcoma Neuropathology in Neurodegenerative Disease

Ian R.A. Mackenzie and Manuela Neumann

Disease Mechanisms of C9ORF72 Repeat Expansions

Tania F. Gendron and Leonard Petrucelli

The Genetics of C9orf72 Expansions

Ilse Gijselinck, Marc Cruts, and Christine Van Broeckhoven

HUNTINGTON AND POLYQ

Huntington's Disease: Mechanisms of Pathogenesis and Therapeutic Strategies

Maria Jimenez-Sanchez, Floriana Licitra, Benjamin R. Underwood, and David C. Rubinsztein

Prion-Like Characteristics of Polyglutamine-Containing Proteins

Margaret M.P. Pearce and Ron R. Kopito

NON-CENTRAL NERVOUS SYSTEM DISEASES

Prion-Like Protein Aggregates and Type 2 Diabetes

Abhisek Mukherjee and Claudio Soto

Noncerebral Amyloidoses: Aspects on Seeding, Cross-Seeding, and Transmission

Gunilla T. Westermark, Marcus Fändrich, Katarzyna Lundmark, and Per Westermark

THERAPEUTICS

Developing Therapeutics for PrP Prion Diseases

Kurt Giles, Steven H. Olson, and Stanley B. Prusiner

Insights from Therapeutic Studies for PrP Prion Disease

Kenta Teruya and Katsumi Doh-ura

Antibody Therapeutics Targeting Aβ and Tau

Gilbert Gallardo and David M. Holtzman

Therapeutic Strategies for Restoring Tau Homeostasis

Zapporah T. Young, Sue Ann Mok, and Jason E. Gestwicki

Glossary

Index

  “This volume is a good resource for scientists in general. Offering information and evidence on prion activity for a variety of disease-implicated proteins, this textbook could be used by a scientist unfamiliar with prion diseases to establish a firm understanding of its principles and parallels to a variety of conditions. Similarly, scientists familiar with the field will find that this publication is a good summary of the vast amount of information that has been published in this rapidly developing (or propagating) field.”
      —The Quarterly Review of Biology