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Prion Diseases

Subject Area(s):  Proteins and ProteomicsInfectious DiseasesHuman Biology and DiseaseDiseases

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

Due June 2017 • 450 pages (approx.), illustrated, index
Hardcover • $135 94.50 (click here to price in UK Pounds)
ISBN  978-1-621820-10-9
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Diseases such as Creutzfeldt–Jakob disease and kuru develop when certain 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 prion-like characteristics, and many of these are associated with important human diseases such as Alzheimer’s and 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 therapeutics. The contributors discuss how proteins such as tau, islet amyloid polypeptide, and α-synuclein aggregate, spread in a prion-like manner, and contribute to various neurodegenerative and extracerebral diseases. Human and animal diseases caused by the prion protein PrP are also covered, as are efforts to develop immunotherapeutics, protein disaggregases, and other drugs to delay or halt their progression.

The authors discuss the development and use of cell culture and animal models of prion diseases, as well as the technologies and small molecules that are being used to study them. This volume is therefore useful for all biomedical scientists and physicians wishing to understand and treat this expanding group of devastating diseases.


Biology and Genetics of PrP Prion Strains
Sina Ghaemmaghami
Prion Strain Diversity
Jason C. Bartz
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
Stuctural Biology of PrP Prions
Gerald Stubbs and Jan Stöhr
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-Binding 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
The Prion-Like Properties of Amyloid-β Assemblies: Implications for Alzheimer’s Disease
Lary C. Walker, Juliane Schelle, and Mathias Jucker
Aβ Prions and the Pathobiology of Alzheimer’s Disease
Joel C. Watts and Stanley B. Prusiner
Structural and Chemical Biology of Presenilin Complexes
Douglas S. Johnson, Yue-Ming Li, Martin Pettersson, and Peter H. St George-Hyslop
Genetics of APP in Alzheimer’s Disease
Julia TCW and Alison M. Goate
Prion Properties of SOD1 in Amyotrophic Lateral Sclerosis and Potential Therapy
Caroline Sibilla and Anne Bertolotti
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
The Transcellular Propagation and Intercellular Trafficking of α-Synuclein
George K. Tofaris, Michel Goedert, and Maria Grazia Spillantini
α-Synuclein: Experimental Pathology
Masato Hasegawa, Takashi Nonaka, and Masami Masuda-Suzukake
Biological Spectrum of ALS Prions
Magdalini Polymenidou and Don W. Cleveland
Clinical Spectrum of Amyotrophic Lateral Sclerosis (ALS)
Leslie I. Grad, Guy A. Rouleau, John Ravits, and Neil R. Cashman
Disease Mechanisms of C9ORF72 Repeat Expansions
Tania F. Gendron and Leonard Petrocelli
The Genetics of C9orf72 Expansions
Ilse Gijselinck, Marc Cruts, and Christine Van Broeckhoven
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
Biology and Pathobiology of TDP-43 and Emergent Therapeutic Strategies
Lin Guo and James Shorter
TDP-43 Prions
Takashi Nonaka and Masato Hasegawa
FUS Neuropathology in Neurodegenerative Disease
Ian R.A. Mackenzie and Manuela Neumann
Noncerebral Amyloidoses: Aspects on Seeding, Cross-Seeding, and Transmission
Gunilla T. Westermark, Marcus Fändrich, Katarzyna Lundmark, and Per Westermark
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