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Transgenetics of Prion Diseases

  • Chapter
Transgenic Models of Human Viral and Immunological Disease

Part of the book series: Current Topics in Microbiology and Immunology ((CT MICROBIOLOGY,volume 206))

Abstract

Prions cause a group of human and animal neurodegenerative diseases which are now classified together because their etiology and pathogenesis involve modification of the prion protein (PrP) (Prusiner 1991). Prion diseases are manifest as infectious, genetic and sporadic disorders (Table 1). These diseases can be transmitted among mammals by the infectious particle designated “prion” (Prusiner 1982). Despite intensive searches over the past three decades, no nucleic acid has been found within prions (Alper et al. 1966, 1967; HUNTER 1972; Riesner et al. 1992); yet, a modified isoform of the host-encoded PrP, designated PrPSc, is essential for infectivity (Büeler et al. 1993; Prusiner 1991; Prusiner et al. 1983, 1993b,c). 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 (Gajdusek 1977, 1985; Sigurdsson 1954).

This Chapter also appears in Vol. 207: Prions Prions Prions (S.B. PRUSINER)

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Authors and Affiliations

  1. Department of Neurology, University of California, HSE-781, San Francisco, CA, 94143-0518, USA

    S. B. Prusiner

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Editors and Affiliations

  1. Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10666 North Torrey Pines Road, La Jolla, CA, 92037, USA

    Francis V. Chisari M.D. (Member, Head) (Member, Head)

  2. Division of Experimental Pathology, The Scripps Research Institute, 10666 North Torrey Pines Road, La Jolla, CA, 92037, USA

    Francis V. Chisari M.D. (Member, Head) (Member, Head)

  3. Department of Neuropharmacology, The Scripps Research Institute, 10666 North Torrey Pines Road, La Jolla, CA, 92037, USA

    Michael B. A. Oldstone M.D. (Member, Head) (Member, Head)

  4. Division of Virology, The Scripps Research Institute, 10666 North Torrey Pines Road, La Jolla, CA, 92037, USA

    Michael B. A. Oldstone M.D. (Member, Head) (Member, Head)

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© 1996 Springer-Verlag Berlin Heidelberg

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Prusiner, S.B. (1996). Transgenetics of Prion Diseases. In: Chisari, F.V., Oldstone, M.B.A. (eds) Transgenic Models of Human Viral and Immunological Disease. Current Topics in Microbiology and Immunology, vol 206. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-85208-4_14

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  • DOI: https://doi.org/10.1007/978-3-642-85208-4_14

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