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Pathogenesis of Prion Diseases

  • Chapter
Protein Misfolding, Aggregation, and Conformational Diseases

Part of the book series: Protein Reviews ((PRON,volume 6))

Abstract

Prion diseases are invariably fatal neurodegenerative disorders affecting humans and many other mammals. Here we discuss the current understanding of prion biology and the pathogenesis of this group of illnesses. We introduce several aspects of prion biology, from the primary structure of the cellular form of the prion protein (PrPC) to the conformational changes that occur during the conversion to the pathologic form, PrPSc. Moreover, we provide an overview of the various prion diseases. We then conclude with the recent discovery of mammalian synthetic prions and the implications that such findings may have to the future of prion research.

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Authors and Affiliations

  1. SISSA - Neurobiology Sector, Ed. Q1 AREA SCIENCE PARK S.S. 14 Km 163, 5 Basovizza, 34012, Trieste, Italy

    Giuseppe Legname

  2. Institute for Neurodegenerative Diseases Department of Pathology, University of California at San Francisco, San Francisco, CA, 94143, USA

    Stephen J. DeArmond

  3. Institute for Neurodegenerative Diseases Department of Cellular and Molecular Pharmacology Department of Biochemistry and Biophysics, University of California at San Francisco, San Francisco, CA, 94143, USA

    Fred E. Cohen

  4. Institute for Neurodegenerative Diseases Department of Neurology Department of Biochemistry and Biophysics, University of California at San Francisco, San Francisco, CA, 94143, USA

    Stanley B. Prusiner

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  1. Department of Biochemistry and Molecular Biology, Center for Computational Biology and Bioinformatics, Indiana University School of Medicine, Indianapolis, IN, 46202

    Vladimir N. Uversky

  2. Institute for Biological Instrumentation, Russian Academy of Sciences, Pushchino, Moscow Region, 142290, Russia

    Vladimir N. Uversky

  3. Department of Chemistry and Biochemistry, University of California, Santa Cruz, CA, 95064, USA

    Anthony L. Fink

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Legname, G., DeArmond, S.J., Cohen, F.E., Prusiner, S.B. (2007). Pathogenesis of Prion Diseases. In: Uversky, V.N., Fink, A.L. (eds) Protein Misfolding, Aggregation, and Conformational Diseases. Protein Reviews, vol 6. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-36534-3_6

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