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Neurogenetics pp 305–314Cite as

Characterization of Prion Proteins

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Part of the book series: Methods in Molecular Biology™ ((MIMB,volume 217))

Abstract

Prion disease represents a group of transmissible neurodegenerative disorders that include scrapie in sheep and goats, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and kuru in humans. The disease is characterized clinically by ataxia, dementia, and myoclonus as well as pathologically by spongiosis, astrocytic gliosis, and neuronal loss (reviewed in refs. 1 and 2). CJD occurs mostly (about 85% of all cases) as sporadic cases (unknown etiology), with the rest of the cases being familial (owing to inheritance of mutations in the prion protein gene) or iatrogenic (due to accidental transmission during medical procedure). Recently, a new variant form of CJD (vCJD) has emerged in the United Kingdom that may originate from the BSE epidemic (3).

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Author information

Authors and Affiliations

  1. Institute of Pathology, Case Western Reserve University and National Prion Disease Pathology Surveillance Center, Cleveland, OH

    Wenquan Zou, Monica Colucci, Pierluigi Gambetti & Shu G. Chen

Authors
  1. Wenquan Zou
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  2. Monica Colucci
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  3. Pierluigi Gambetti
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  4. Shu G. Chen
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Editor information

Editors and Affiliations

  1. University of Tennessee Medical Center, Knoxville, Tennessee, USA

    Nicholas T. Potter

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© 2003 Humana Press Inc.

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Zou, W., Colucci, M., Gambetti, P., Chen, S.G. (2003). Characterization of Prion Proteins. In: Potter, N.T. (eds) Neurogenetics. Methods in Molecular Biology™, vol 217. Springer, Totowa, NJ. https://doi.org/10.1385/1-59259-330-5:305

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  • DOI: https://doi.org/10.1385/1-59259-330-5:305

  • Publisher Name: Springer, Totowa, NJ

  • Print ISBN: 978-0-89603-990-2

  • Online ISBN: 978-1-59259-330-9

  • eBook Packages: Springer Protocols

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