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Bovine Spongiform Encephalopathy pp 202–231Cite as

Deciphering Prion Diseases with Transgenic Mice

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

Abstract

Prions cause a group of human and animal neurodegenerative diseases that are now classified together because their etiology and pathogenesis involve modification of the prion protein (PrP) (1). Prion diseases are manifest as infectious, genetic, and sporadic disorders (Table 16.1). These diseases can be transmitted among mammals by the infectious particle designated “prion” (2). Despite intensive searches over the past three decades, no nucleic acid has been found within prions (3–6), yet a modified isoform of the host-encoded PrP designated PrPSc is essential for infectivity (1, 7–10). In fact, considerable experimental data argue that prions are composed exclusively of PrPSc. Earlier terms used to describe the prion diseases include transmissible encephalopathies, spongiform encephalopathies, and slow virus diseases (11–13).

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Authors
  1. Glenn C. Telling
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  2. Michael Scott
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  3. Stanley B. Prusiner
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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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Telling, G.C., Scott, M., Prusiner, S.B. (1996). Deciphering Prion Diseases with Transgenic Mice. 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_16

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  • DOI: https://doi.org/10.1007/978-1-4612-2406-8_16

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4612-7527-5

  • Online ISBN: 978-1-4612-2406-8

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