Prion Protein

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). 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.

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