Prion Diseases

Subject Area(s): Proteins and Proteomics; Infectious Diseases; Human Biology and Disease; Diseases

Edited by Stanley B. Prusiner, University of California, San Francisco

Download a Free Excerpt from Prion Diseases:

Introduction
Preface
Sporadic and Infectious Human Prion Diseases
Index

© 2017 • 677 pages, illustrated (83 color and 34 B&W), index
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ISBN 978-1-621822-83-7
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Description
Contents
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Description

Diseases such as Creutzfeldt-Jakob disease and kuru develop when PrP proteins form prions by misfolding, clumping together, and spreading from cell to cell. Over the past decade, a number of proteins have been reported to possess the characteristics of PrP prions. A growing collection of disorders has been found to be caused by prions; some of these prion diseases include such devastating illnesses as Alzheimer's and Parkinson's and possibly type 2 diabetes.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Medicine covers the progress that has recently been made in our understanding of the pathophysiology of prion diseases, as well as ongoing efforts to develop effective therapeutics. The contributors discuss how proteins such as tau, islet amyloid polypeptide, and α-synuclein adopt alternative shapes that lead them to aggregate, resulting in cellular degeneration. Therapies for human and animal diseases caused by prions are also covered. This volume is therefore useful for all biomedical scientists and physicians wishing to understand and treat this expanding group of devastating disorders.

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

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Description

Contents

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