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Molecular Pathology of the Prions pp 71–83Cite as

Characterization of Bovine Spongiform Encephalopathy and Scrapie Strains/Isolates by Immunochemical Analysis of PrPSc

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Part of the book series: Methods in Molecular Medicine™ ((MIMM,volume 59))

Abstract

In the past two decades, thoroughly standardized mouse incubation time and brain lesion profile scoring assays have been developed to discriminate between prion strains. However, in these mouse infection experiments, large numbers of animals (about 20 mice/line) from three different highly inbred mouse lines (C57Bl, VM95, RIII), plus their intercrosses, need to be infected, and their brain tissues subsequently examined (1-8). Although results obtained are highly reliable, the effort and time needed for conducting these experiments are considerable. Therefore, alternative criteria and techniques have been developed to characterize transmissible spongiform encephalopathy (TSE) agents. Prion infections are accompanied by the accumulation of an abnormal isoform (designated PrPSc) of normal host-encoded prion protein (PrPC). Both isoforms have the same amino acid sequence and molecular mass, but differ significantly in their three-dimensional structure and biochemical characteristics. The three-dimensional structure of PrPC is characterized by a high ?-helical content (9); all or part of it undergoes a posttranslational modification to β-sheet in PrPSc (10-12). Although PrPC (33-35 kDa) is completely hydrolyzed by protease treatment, PrPSc is partially resistant to proteinase K(PK) as 62 N-terminal amino acids are cleaved, leaving a core fragment of approx 141 amino acids (27-30 kDa) unhydrolyzed (13).

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Author information

Authors and Affiliations

  1. Federal Research Centre for Virus Diseases of Animals, Tuebingen, Germany

    Martin H. Groschup, Frauke Junghans & Martin Eiden

  2. Behörde für Arbeit, Gesundheit und Soziales Amt für Gesundheit, Hygiene-Institut Hamburg, Hamburg, Germany

    Thorsten Kuczius

Authors
  1. Martin H. Groschup
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  2. Frauke Junghans
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  3. Martin Eiden
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  4. Thorsten Kuczius
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Editor information

Editors and Affiliations

  1. Department of Experimental Psychology, MRC Comparative Cognition Team, School of Clinical Veterinary Medicine, Cambridge, UK

    Harry F. Baker

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© 2001 Humana Press Inc.

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Groschup, M.H., Junghans, F., Eiden, M., Kuczius, T. (2001). Characterization of Bovine Spongiform Encephalopathy and Scrapie Strains/Isolates by Immunochemical Analysis of PrPSc. In: Baker, H.F. (eds) Molecular Pathology of the Prions. Methods in Molecular Medicine™, vol 59. Humana Press. https://doi.org/10.1385/1-59259-134-5:71

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  • DOI: https://doi.org/10.1385/1-59259-134-5:71

  • Publisher Name: Humana Press

  • Print ISBN: 978-0-89603-924-7

  • Online ISBN: 978-1-59259-134-3

  • eBook Packages: Springer Protocols

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