MRI detects therapeutic effects in weanling Niemann-Pick type C mice

Silvia Lope-Piedrafita, John W. Totenhagen, Chad M. Hicks, Robert P. Erickson, Theodore P. Trouard, Theodore P. Trouard

    Research output: Contribution to journalArticleResearchpeer-review

    18 Citations (Scopus)


    To noninvasively evaluate the early effects of Niemann-Pick type C (NPC) disease, diffusion tensor imaging (DTI) was carried out in the brains of very young (23-day-old) mice. The diffusion of water in white matter tracts of Npc1-/- mice at this young age was already abnormal, exhibiting decreased anisotropy, as quantified by fractional anisotropy (FA), compared with their wild-type littermates, the controls. Postmortem histological staining revealed myelin de.ciencies in Npc1-/- mice, consistent with the reduction in FA measured vivo. Beneficial effects of treatment with allopregnanolone and/ or 2 hydroxypropyl-beta-cyclodextrin was also detectable at this age by FA, which correlated with increased myelination as seen by histology. This is the earliest detection of a therapeutic effect in Npc1-/- mice. © 2008 Wiley-Liss, Inc.
    Original languageEnglish
    Pages (from-to)2802-2807
    JournalJournal of Neuroscience Research
    Publication statusPublished - 8 Dec 2008


    • Allopregnanolone
    • Beta-cyclodextrin
    • Brain
    • DTI
    • Dysmyelination
    • Mouse
    • Neurodegeneration
    • Neurosteroids


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