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 journalArticlepeer-review

19 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)2802-2807
Number of pages6
JournalJournal of Neuroscience Research
Issue number12
StatePublished - 2008


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

ASJC Scopus subject areas

  • Cellular and Molecular Neuroscience


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