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    Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy

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    FowlerS_JCM_1(1)1.pdf (2.775Mb)
    Issue Date
    2012-01-19
    Author
    Reddy, Adarsh S.
    Wozniak, David F.
    Farber, Nuri B.
    Dearborn, Joshua T.
    Fowler, Stephen C.
    Sands, Mark S.
    Publisher
    MDPI
    Type
    Article
    Article Version
    Scholarly/refereed, publisher version
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    Abstract
    Tremor is a prominent phenotype of the twitcher mouse, an authentic genetic model of Globoid-Cell Leukodystrophy (GLD, Krabbe’s disease). In the current study, the tremor was quantified using a force-plate actometer designed to accommodate low-weight mice. The actometer records the force oscillations caused by a mouse’s movements, and the rhythmic structure of the force variations can be revealed. Results showed that twitcher mice had significantly increased power across a broad band of higher frequencies compared to wildtype mice. Bone marrow transplantation (BMT), the only available therapy for GLD, worsened the tremor in the twitcher mice and induced a measureable alteration of movement phenotype in the wildtype mice. These data highlight the damaging effects of conditioning radiation and BMT in the neonatal period. The behavioral methodology used herein provides a quantitative approach for assessing the efficacy of potential therapeutic interventions for Krabbe’s disease.
    Description
    This is the publisher's version, also available electronically from "http://www.mdpi.com".
    URI
    http://hdl.handle.net/1808/17677
    DOI
    https://doi.org/10.3390/jcm1010001
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    • Pharmacy Scholarly Works [261]
    Citation
    Reddy, A. S., Wozniak, D. F., Farber, N. B., Dearborn, J. T., Fowler, S. C. & Sands, M. S. (2012) Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy. Journal of Clinical Medicine, 1(1), 1-14. http://www.dx.doi.org/10.3390/jcm1010001

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    KU Libraries
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    Lawrence, KS 66045
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    Contact KU ScholarWorks
    785-864-8983
    KU Libraries
    1425 Jayhawk Blvd
    Lawrence, KS 66045
    785-864-8983

    KU Libraries
    1425 Jayhawk Blvd
    Lawrence, KS 66045
    Image Credits
     

     

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