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Conformation as Therapeutic Target in the Prionoses and Other Neurodegenerative Conditions

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Book cover Molecular Pathology of the Prions

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

Abstract

Neurodegenerative conditions are increasing in prevalence as the average human life expectancy rises. Alzheimer’s disease (AD) is the fourth commonest cause of death in the United States; the recent outbreak of new variant Creutzfeldt-Jakob disease (nvCJD) has raised the specter of a large population being at risk to develop this prionosis. The pathogenesis of many neurodegenerative diseases is now recognized to be associated with abnormalities of protein conformation. A common theme in these disorders is the conversion of a soluble normal precursor protein into an insoluble, aggregated, ?-sheet rich form that is toxic. In AD, a critical event is the conversion of the normal, soluble A? (sA?) peptide into fibrillar A?, within neuritic plaques and congophilic angiopathy (1). Similarly, in the prionoses, the central event is the conversion of the normal prion protein, PrPC, to PrPSc (2). An increased ?-sheet content characterizes both A? and PrPSc.

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Author information

Authors and Affiliations

  1. Departments of Neurology and Pathology, New York University Medical Center, New York, NY

    Thomas Wisniewski & Einar M. Sigurdsson

  2. Hopital Necker, Paris, France

    Pierre Aucouturier

  3. Departments of Neurology and Pathology, New York University Medical Center, New York, NY

    Blas Frangione

Authors
  1. Thomas Wisniewski
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  2. Einar M. Sigurdsson
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  3. Pierre Aucouturier
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  4. Blas Frangione
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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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Wisniewski, T., Sigurdsson, E.M., Aucouturier, P., Frangione, B. (2001). Conformation as Therapeutic Target in the Prionoses and Other Neurodegenerative Conditions. In: Baker, H.F. (eds) Molecular Pathology of the Prions. Methods in Molecular Medicine™, vol 59. Humana Press. https://doi.org/10.1385/1-59259-134-5:223

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  • DOI: https://doi.org/10.1385/1-59259-134-5:223

  • Publisher Name: Humana Press

  • Print ISBN: 978-0-89603-924-7

  • Online ISBN: 978-1-59259-134-3

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