Abstract
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 language | English |
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Pages (from-to) | 2802-2807 |
Journal | Journal of Neuroscience Research |
Volume | 86 |
DOIs | |
Publication status | Published - 8 Dec 2008 |
Keywords
- Allopregnanolone
- Beta-cyclodextrin
- Brain
- DTI
- Dysmyelination
- Mouse
- Neurodegeneration
- Neurosteroids