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Prion Protein

1st Edition - August 21, 2017

Editors: Giuseppe Legname, Silvia Vanni

Hardback ISBN:

9 7 8 - 0 - 1 2 - 8 1 1 2 2 6 - 7

eBook ISBN:

9 7 8 - 0 - 1 2 - 8 1 1 2 2 7 - 4

Prion Protein, Volume 150, the latest volume in the Progress in Molecular Biology and Translational Science series, focuses on Prion Protein, a protein that is considered to be… Read more

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Prion Protein, Volume 150, the latest volume in the Progress in Molecular Biology and Translational Science series, focuses on Prion Protein, a protein that is considered to be the archetype of intrinsically disordered proteins. This updated volume includes comprehensive sections on a variety of timely topics, including Functions of Prion Protein, Copper and Prion protein, Cell Biology of Prion Protein, Structural Studies of Prion Proteins, Molecular Simulations on Wild Type and Mutant Prion Proteins, Genetics of Prion Protein, The Prion Concept and Synthetic Prions, and Transgenic Mouse Models.

As neurodegenerative diseases represent a health issue that is receiving increasing attention from the scientific community due to their social and economic impact, this series is an ideal resource for the latest research in molecular biology and translational science.

1. Functions of the Prion Protein
Théo Z. Hirsch, Séverine Martin-Lannerée and Sophie Mouillet-Richard

2. Copper- and Zinc-Promoted Interdomain Structure in the Prion Protein: A Mechanism for Autoinhibition of the Neurotoxic N-Terminus
Eric G.B. Evans and Glenn L. Millhauser

3. Cell Biology of Prion Protein
Daniela Sarnataro, Anna Pepe and Chiara Zurzolo

4. Understanding the Effect of Disease-Related Mutations on Human Prion Protein Structure: Insights From NMR Spectroscopy
Ivana Biljan, Gregor Ilc and Janez Plavec

5. Structural Modeling of Human Prion Protein’s Point Mutations
Giulia Rossetti and Paolo Carloni

6. Prion Protein and Genetic Susceptibility to Diseases Caused by Its Misfolding
George A. Carlson

7. The Prion Concept and Synthetic Prions
Giuseppe Legname and Fabio Moda

8. Gene Targeted Transgenic Mouse Models in Prion Research
Abigail B. Diack, James D. Alibhai and Jean C. Manson

9. Transmission and Replication of Prions
A. Marín-Moreno, N. Fernández-Borges, J.C. Espinosa, O. Andréoletti and J.M. Torres

10. Immunology of Prion Protein and Prions
Neil A. Mabbott

11. Phenotypical Variability in Bovine Spongiform Encephalopathy: Epidemiology, Pathogenesis, and Diagnosis of Classical and Atypical Forms
Cristiano Corona, Elena V. Costassa, Barbara Iulini, Elena Bozzetta, Maria Mazza, Rosanna Desiato, Giuseppe Ru and Cristina Casalone

12. Scrapie, CWD, and Transmissible Spongiform Encephalopathy
Candace K. Mathiason

13. Infectious and Sporadic Prion Diseases
Richard Knight

14. Neuropathology of Human Prion Diseases
Diane L. Ritchie and James W. Ironside

15. The Structure of the Infectious Prion Protein and Its Propagation
Jesús R. Requena and Holger Wille

16. Protein Misfolding Cyclic Amplification of Infectious Prions
Fabio Moda

17. Amplified Detection of Prions and Other Amyloids by RT-QuIC in Diagnostics and the Evaluation of Therapeutics and Disinfectants
Byron Caughey, Christina D. Orru, Bradley R. Groveman, Andrew G. Hughson, Matteo Manca, Lynne D. Raymond, Gregory J. Raymond, Brent Race, Eri Saijo and Allison Kraus

18. Biochemical Characterization of Prions
Michele Fiorini, Matilde Bongianni, Salvatore Monaco and Gianluigi Zanusso

19. Omics of Prion Diseases
Silvia Vanni

20. Therapeutic Approaches to Prion Diseases
Annachiara Gandini and Maria L. Bolognesi

21. Biosafety of Prions
Edoardo Bistaffa, Martina Rossi, Chiara M.G. De Luca and Fabio Moda

Giuseppe Legname

Giuseppe Legname carried undergraduate and postgraduate studies in biochemistry and molecular biology at the Università degli Studi of Milan, Italy. He gained his Doctor of Philosophy (D.Phil.) degree in Biological Sciences at the University of Warwick in 1997. After a long spell in Industry where he coauthored many scientific papers and patents in the field of immunotherapy, he moved to academia at the National Institute for Medical Research (NIMR), Medical Research Council in London, UK.

In 1999 he joined the faculty of the Department of Neurology at the University of California at San Francisco (UCSF), California, USA.

During the seven-and-half years at UCSF he was involved in basic research projects in the field of Prion Biology and Disease at the Institute for Neurodegenerative Diseases (IND), under the direction of 1997 Nobel Laureate Professor Stanley B. Prusiner.

Since December 2006, he has joined the faculty of the Scuola Internazionale Superiore di Studi Avanzati (SISSA), in Trieste, where he is currently managing a new Prion Biology Laboratory.

At IND he was responsible for several Projects and Science Cores of funded National Health Institute (NIH) grants on prions. During his scientific experience in the USA he has coauthored several seminal papers, book chapters and patents. At SISSA, Giuseppe Legname has joined the Neuroscience Department and the main focus of his research program is in the field of mammalian Prion Biology (physiological function of the prion protein in mammals) and Prion Disease (mechanisms of prion replication and structural characterization of molecular determinants for prion infectivity).

Affiliations and expertise

Scuola Internazionale Superiore di Studi Avanzati, Trieste, Italy

Silvia Vanni

Silvia Vanni achieved a Bachelor of Science (B.Sc.) in Biotechnology in 2008 and a Master of Science (M.Sc.) in Medical Biotechnology cum laude in in 2011, both at the University of Bologna, Italy.

She obtained her Philosophy Doctoral (Ph.D.) degree in Functional and Structural Genomic at the Scuola Internazionale Superiore di Studi Avanzati (SISSA) of Trieste, Italy in 2015, under the supervision of Prof. Giuseppe Legname.

During the Ph.D. course she focused her main efforts in understanding the molecular events underlying the onset and progression of human prion diseases.

She is currently working as a post-doc in Prof. Legname’s lab at SISSA, and her main project is about investigating the gene expression alterations among different tissues in various neurodegenerative diseases with the aim of identifying potential biomarkers for early diagnosis.

Affiliations and expertise

Scuola Internazionale Superiore di Studi Avanzati, Trieste, Italy