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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

Due August 2017 • 350 pages (approx.), illustrated, index
Hardcover • £84 (click here to price in US Dollars)
ISBN  978-1-621820-93-2

This title also available in: Paperback

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Prions are best known as the agents of mad cow and related diseases, and a growing number of proteins with prion-like properties have been implicated in diseases such as Parkinson’s. But some recently discovered prion-like proteins do not appear to induce pathological changes and, in fact, may be key players 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 and prion-like proteins in health and disease. The contributors review the structures of prions and prion-like proteins, how they aggregate into amyloid fibrils and other insoluble complexes, and the unique biological properties of the aggregated forms (e.g., infectivity, gain/loss of function, and self-templating). The normal physiological roles of prion-like proteins (e.g., CPEB in the brain and MAVS in the immune response) are discussed, as is the formation of abnormal protein aggregates via prion-like mechanisms in various human diseases.

The authors also discuss prions in yeast and other fungi, which are convenient models for studying mechanisms of amyloid formation and propagation. This volume is therefore an essential reference for biochemists, cell and molecular biologists, and all who wish to understand how these alternatively folded, self-propagating proteins function.


Widening Spectrum of Prion Biology and Diseases
Stanley B. Prusiner
Experimental Approaches to Neurodegeneration
Stanley B. Prusiner and Joel Watts
The HET-S/s Prion Motif in the Control of Programmed Cell Death
Roland Riek and Sven J. Saupe
The Amyloid Phenomenon and its Links with Human Disease
Christopher M. Dobson
Protein Quality Control in Health and Disease
Tatyana Dubnikov, Tziona Ben-Gedalya, and Ehud Cohen
Yeast and Fungal Prions
Reed B. Wickner
Prions, Chaperones, and Proteostasis in Yeast
Tatiana A. Chernova, Keith D. Wilkinson, and Yury O. Chernoff
The 3D Structures of Amyloids
Roland Riek
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
Cross-β Polymerization of Low Complexity Sequence Domains
Masato Kato and Steven L. McKnight
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
Molecular Genetics of Neurodegenerative Dementias
Flora I. Hinz and Daniel H. Geschwind
Tau PET Imaging
Hartmuth C. Kolb and José Ignacio Andrés
Potential Pathways of Abnormal Tau and α-Synuclein Dissemination in Sporadic Alzheimer’s and Parkinson’s Diseases
Heiko Braak and Kelly Del Tredici
Inactivation of Prions—Deep Brain Stimulation and Synuclein Prions
Kurt Giles and Stanley B. Prusiner