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    Accumulation of glial fibrillary acidic protein and histone H4 in brain storage bodies of Tibetan terriers with hereditary neuronal ceroid lipofuscinosis

    Katz, Martin L.
    Sanders, Douglas N., 1973-
    Mooney, Brian P.
    Johnson, Gary S.
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    [PDF] AccumulationGlialFibrillaryAcidicProtein.pdf (708.4Kb)
    Date
    2007
    Format
    Article
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    Abstract
    The neuronal ceroid lipofuscinoses (NCLs) are inherited neurodegenerative diseases characterized by massive accumulation of autofluorescent storage bodies in neurons and other cells. A late-onset form of NCL occurs in Tibetan terrier dogs. Gel electrophoretic analyses of isolated storage body proteins from brains of affected dogs indicated that a protein of approximately 50 kDa was consistently prominent and a 16 kDa component was present in some brain storage body preparations. Mass spectral analysis identified the 50 kDa protein as glial fibrillary acidic protein (GFAP), isoform 2. GFAP identification was supported by immunoblot and immunohistochemical analyses. Histone H4 was the major protein in the 16 kDa component. Specific accumulation of GFAP and histone H4 in storage bodies has not been previously reported for any of the NCLs. Tibetan terrier NCL may be the canine correlate of one of the human adult-onset NCLs for which the genetic bases and storage body compositions have not yet been determined.
    URI
    http://hdl.handle.net/10355/3246
    Part of
    Proteomics Center publications (MU)
    Citation
    Journal of Inherited Metabolic Disease, Volume 30, Number 6 (November, 2007)
    Rights
    OpenAccess
    This work is licensed under a Creative Commons Attribution-NonCommerical-NoDerivs 3.0 License.
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    • Proteomics Center publications (MU)

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