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Prion Biology

Subject Area(s):  Cell BiologyProteins and ProteomicsHuman Biology and Disease

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

Download a Free Excerpt from Prion Biology:

Functional Prions in the Brain

© 2017 • 456 pages, illustrated (74 color and 16 B&W), index
Hardcover •
ISBN  978-1-621820-93-2

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Prions are best known as the infectious proteins causing mad cow and related diseases, and a growing number of proteins with similar properties are implicated in disorders such as Alzheimer’s and Parkinson’s. But some recently discovered prion proteins do not seem to induce pathological changes and, in fact, may function 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 in health and disease. The contributors review the structures of prion proteins and how they adopt alternative structures and aggregate into amyloid fibrils and other insoluble complexes. The alternatively folded prion forms display unique biological properties. Although some prion proteins feature in cellular dysfunction, others have normal physiological roles (e.g., CPEB in the brain and MAVS in the immune response). This volume is an essential reference for biochemists, cell and molecular biologists, and all who wish to understand how prions are formed from alternatively folded, self-propagating proteins.


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
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
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-β Polymerization of Low Complexity Sequence Domains
Masato Kato and Steven L. McKnight
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 α-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. Soares, Luciana P. Rangel, and Jerson L. Silva


review:  “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

Related Titles


Prion Diseases [Paperback]