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    Therapeutic interventions ameliorating prion disease

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    Author
    Brazier, MW; Wall, VA; Brazier, BW; Masters, CL; Collins, SJ
    Date
    2009-02-01
    Source Title
    EXPERT REVIEW OF ANTI-INFECTIVE THERAPY
    Publisher
    TAYLOR & FRANCIS LTD
    University of Melbourne Author/s
    WALL, VANESSA; Masters, Colin; Collins, Steven; BRAZIER, MARCUS
    Affiliation
    Pathology
    Metadata
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    Document Type
    Journal Article
    Citations
    Brazier, M. W., Wall, V. A., Brazier, B. W., Masters, C. L. & Collins, S. J. (2009). Therapeutic interventions ameliorating prion disease. EXPERT REVIEW OF ANTI-INFECTIVE THERAPY, 7 (1), pp.83-105. https://doi.org/10.1586/14787210.7.1.83.
    Access Status
    This item is currently not available from this repository
    URI
    http://hdl.handle.net/11343/25654
    DOI
    10.1586/14787210.7.1.83
    Description

    C1 - Journal Articles Refereed

    Abstract
    Of the many unresolved issues in relation to prion diseases, effective treatments remain an elusive exigency, although some progress has been made. This review describes disease-ameliorating therapeutic strategies reported to date in animal models of prion disease, as well as providing a brief overview of selected completed human treatment trials. Included in vivo studies have been broadly dichotomized according to the time of introduction of the treatment in relation to animal inoculation and also according to their possible principal mechanism of action, although the latter is not always entirely clear, and often there is likely to be more than one mechanism. Consequent to the pathogenic primacy of cellular prion protein (PrP(c))-to-scrapie PrP(c) (PrP(sc)) conversion, most reported treatments appear to directly target this replication process, although various other strategies, such as depletion of reaction substrates and abrogation of downstream effector pathways, have been utilized. Many factors, including experimental design, militate against reliable extrapolation of study results to the routine clinical setting or limit easy translational application to human disease. Notably problematic are approaches wherein benefit has been shown but the treatment was initiated before, at or soon after inoculation of experimental animals.
    Keywords
    Clinical Microbiology; Microbiology not elsewhere classified; Nervous System and Disorders; Nervous System and Disorders

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