The Prion Phenomena in Neurodegenerative Diseases: New Frontiers in Neuroscience


Giuseppe Legname, PhD and Gabriele Giachin, PhD (Editors)
SISSA via Bonomea, Trieste, Italy

Series: Neurodegenerative Diseases – Laboratory and Clinical Research
BISAC: MED056000

The most fascinating and unique feature of prion diseases is that they are caused almost exclusively by a proteinaceous and infectious particle termed prions by the Nobel Prize laureate S.B. Prusiner, who discovered this class of pathogens. In the latter part of the 1990s, mad-cow disease, a disease caused by prions acquired through foodborne transmission, raised unprecedented public concern due to the concrete possibility that prions in animals could be transmitted to humans through the food chain. For roughly two decades, prions were under intense scrutiny and many studies were undertaken worldwide.

These investigations have led our community to a better risk assessment and management of prion diseases in humans and in animals, substantially limiting the possibility of new prion epidemics. Nowadays, prions have been brought once again to the foreground after the discovery that a variety of neurodegenerative diseases, in particular Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis, share fundamental features with prions, including protein misfolding and aggregation in the brain, cell-to-cell transmission and in vivo infectivity.

Therefore, studying prions might help to understand the pathological mechanism of these disorders. The Prion Phenomena In Neurodegenerative Diseases: New Frontiers in Neuroscience is a book that benefits from the contribution of leading scientists in different fields of neuroscience, including Gianluigi Zanusso, Holger Wille, Fabrizio Tagliavini, Andrew F. Hill, Jerson L. Silva, Vladimir N. Uversky, Henrike Heise, David W. Colby, Neil R. Cashman and the Nobel Prize laureate Eric R. Kandel. This chapter collection discusses the development of prions and their various diseases, and provides a detailed overview about the state of the art of the novel prion phenomena observed in other fatally damaging protein misfolding disorders. This book represents an up-to-date review of different protein-misfolding diseases, serving as an invaluable tool for both specialized researchers working in the field of neurodegeneration and for a broad spectrum of academic readers that wish to learn more about the prion phenomena.
(Imprint: Nova Biomedical)

Table of Contents

Table of Contents


About the Editors

List of Contributors

Neuropathological Phenotypes in Human Prion Disorders
(Gianluigi Zanusso, Sergio Ferrari and Salvatore Monaco, Department of Neurological and Movement Sciences, University of Verona, Verona, Italy)

X-Ray Fiber Diffraction from Prions: Of Mice and Beta-Solenoids
(Holger Wille, Centre for Prions and Protein Folding Diseases and Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada)
Free Download Available

Neurotoxicity in Prion Disease: Relationships with other Neurodegenerative Diseases and Outlooks for Therapeutic Intervention
(Cathryn L. Ugalde, Victoria A. Lawson, David I. Finkelstein and Andrew F. Hill, Department of Biochemistry and Molecular Biology, Bio21 Molecular Science & Biotechnology Institute, The University of Melbourne, Parkville, Victoria, Australia, and others)

Glycosaminoglycans in Prion and Prion-like Diseases
(Tuane C.R.G. Vieira and Jerson L. Silva, Instituto Federal do Rio de Janeiro, Rio de Janeiro, Brazil, and others)
Free Download Available

Complexity of Aggregation Pathway of α-synuclein
(Leonid Breydo and Vladimir N. Uversky, Department of Molecular Medicine, Byrd Alzheimer’s Research Institute, Morsani College of Medicine, University of South Florida, Tampa, FL, USA, and others)

Structural Characterization of α-synuclein Amyloids
(Wolfgang Hoyer, Hamed Shaykhalishahi, Santhosh Ayalur-Karunakaran and Henrike Heise, Heinrich Heine University Düsseldorf, Institute of Physical Biology, Universitätsstraße, Düsseldorf, Germany, and others)

Prion-Like Spreading of Aβ and tau Aggregates and their Relevance as Drivers of Pathology in Alzheimer’s Disease
(Giuseppe Di Fede, Marcella Catania and Fabrizio Tagliavini, Fondazione IRCSS “Carlo Besta” Neurological Institute, Division of Neurology V and Neuropathology, Milan, Italy)

Tau Strains and their Propagation in Experimental Disease Models
(Kyle P. McHugh, Olga A. Morozova and David W. Colby, Department of Chemical and Biomolecular Engineering, University of Delaware, Newark, DE, USA)

Prion-Like Mechanisms in Amyotrophic Lateral Sclerosis
(Leslie I. Grad, Edward Pokrishevsky and Neil R. Cashman, Department of Medicine (Neurology), Djavad Mowafaghian Centre for Brain Health, University of British Columbia, Vancouver BC, Canada)
Free Download Available

Prions in Yeast: Thinking Outside of the Brain
(Polina Drozdova and Andrew Matveenko, Dept. of Genetics and Biotechnology, Saint Petersburg State University, St. Petersburg, Russia, and others)

Functional Prions: The Case Study of Aplysia
(Eric R. Kandel, Zuckerman Mind Brain Behavior Institute, Department of Neuroscience, Kavli Institute for Brain Science, Howard Hughes Medical Institute
College of Physicians and Surgeons of Columbia University, New York State Psychiatric Institute, New York, NY, USA)

Concluding Remarks
(Giuseppe Legname, Suzana Aulić and Gabriele Giachin, Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati (SISSA), Trieste, Italy, and others)


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