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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

Download a Free Excerpt from Prion Diseases:

Sporadic and Infectious Human Prion Diseases

© 2017 • 677 pages, illustrated (83 color and 34 B&W), index
Paperback • $79 55.30
ISBN  978-1-621822-83-7
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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.


An Introduction to Prion Diseases
Stanley B. Prusiner
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
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
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
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'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
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
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


review:  “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

Related Titles


Prion Biology [Hardcover]