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    • University of Missouri-Columbia
    • Graduate School - MU Theses and Dissertations (MU)
    • Theses and Dissertations (MU)
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    • 2009 Dissertations (MU)
    • 2009 MU dissertations - Access restricted to MU
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    Characterization of cellular pathways in spinal muscular atrophy

    Rose, Ferrill Franklin, 1978-
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    [PDF] research.pdf (4.100Mb)
    Date
    2009
    Format
    Thesis
    Metadata
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    Abstract
    [ACCESS RESTRICTED TO THE UNIVERSITY OF MISSOURI AT REQUEST OF AUTHOR.] Spinal Muscular Atrophy (SMA) is an autosomal recessive genetic disease, and is the leading genetic cause of death in infants. SMA is a severe neuromuscular disease characterized by loss of spinal [alpha]-motor neurons, resulting in the paralysis of skeletal muscle. SMA is caused by deficiency of Survival Motor Neuron (SMN) protein levels. Currently there is no effective treatment for SMA. The work presented in this thesis characterizes several potential SMA therapeutic targets. Follistatin is a natural antagonist of myostatin and over-expression of follistatin in mouse muscle leads to profound increases in skeletal muscle mass. To determine whether enhanced muscle mass impacts SMA, we administered recombinant follistatin to a SMA mouse model. Treated animals exhibited increased mass in several muscle groups, elevation in the number and cross-sectional area of ventral horn cells, gross motor function improvement, and mean lifespan extension by 30%, by preventing some of the early deaths, as compared to control animals. Reversing muscle atrophy associated with SMA may represent an unexploited therapeutic target for the treatment of SMA. The Wallerian degeneration slow (Wlds) gene is a spontaneous dominant mutation in mice that delays axon degeneration by approximately 2-3 weeks. We set out to examine the effect of Wlds on the phenotype of a mouse model of SMA. We found that Wlds does not alter the SMA phenotype, indicating that Wallerian degeneration does not directly contribute to the pathogenesis of SMA development.
    URI
    https://hdl.handle.net/10355/6742
    https://doi.org/10.32469/10355/6742
    Degree
    Ph. D.
    Thesis Department
    Microbiology (Medicine) (MU)
    Rights
    Access is limited to the campus of the University of Missouri--Columbia.
    Collections
    • 2009 MU dissertations - Access restricted to MU
    • Molecular Microbiology and Immunology electronic theses and dissertations (MU)

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