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Cellular and Scrapie Prion Protein Immunolocalization and In Vitro Amyloid Formation

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Bovine Spongiform Encephalopathy

Part of the book series: Serono Symposia USA Norwell, Massachusetts ((SERONOSYMP))

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Abstract

Spongiform encephalopathies in humans and animals include fatal neurodegenerative disorders that can be sporadic, infectious, or inherited (1). Regardless of their origin, spongiform encephalopathies are all transmissible to an appropriate animal species (2), with the exception of fatal familial insomnia (FFI) (3), whose transmission is still unknown. The unique features of the infectious agent that causes spongiform encephalopathies prompted Prusiner (4) to coin the term prion to define a new category of pathogens. In Prusiner’s terminology, the etiologic definition is substituted for the neuropathologic one, and spongiform encephalopathies are called prion diseases. Considerable neuropathologic variability among this group of diseases has been described (5). Some forms are characterized by widespread spongiform changes, while others show mostly prion protein (PrP) amyloid deposition (5). Scrapie, a disease naturally occurring in sheep and goats, is the prototype of prion diseases and has been adapted to various experimental rodent species.

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Authors
  1. Mauro Ceroni
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  2. Jiri Safar
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  3. Pedro Piccardo
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  4. Paul P. Liberski
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  5. Paola Pergami
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  6. Clarence J. Gibbs Jr.
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Editors and Affiliations

  1. Laboratory of Central Nervous System Studies Basic Neurosciences Program Division of Intramural Research, National Institute of Neurological Disorders and Stroke National Institutes of Health, Bethesda, MD, 20892-4122, USA

    Clarence J. Gibbs Jr. Ph.D.

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© 1996 Springer-Verlag New York, Inc.

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Ceroni, M., Safar, J., Piccardo, P., Liberski, P.P., Pergami, P., Gibbs, C.J. (1996). Cellular and Scrapie Prion Protein Immunolocalization and In Vitro Amyloid Formation. In: Gibbs, C.J. (eds) Bovine Spongiform Encephalopathy. Serono Symposia USA Norwell, Massachusetts. Springer, New York, NY. https://doi.org/10.1007/978-1-4612-2406-8_25

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  • DOI: https://doi.org/10.1007/978-1-4612-2406-8_25

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