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 journalArticle

16 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)2802-2807
Number of pages6
JournalJournal of Neuroscience Research
Volume86
Issue number12
DOIs
StatePublished - 2008

Fingerprint

Anisotropy
Therapeutic Uses
Type C Niemann-Pick Disease
Pregnanolone
Diffusion Tensor Imaging
Myelin Sheath
Histology
Staining and Labeling
Water
Brain
Therapeutics

Keywords

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

ASJC Scopus subject areas

  • Cellular and Molecular Neuroscience

Cite this

Lope-Piedrafita, S., Totenhagen, J. W., Hicks, C. M., Erickson, R. P., Trouard, T. P., & Trouard, T. P. (2008). MRI detects therapeutic effects in weanling Niemann-Pick type C mice. Journal of Neuroscience Research, 86(12), 2802-2807. https://doi.org/10.1002/jnr.21707

MRI detects therapeutic effects in weanling Niemann-Pick type C mice. / Lope-Piedrafita, Silvia; Totenhagen, John W.; Hicks, Chad M.; Erickson, Robert P.; Trouard, Theodore P.; Trouard, Theodore P.

In: Journal of Neuroscience Research, Vol. 86, No. 12, 2008, p. 2802-2807.

Research output: Contribution to journalArticle

Lope-Piedrafita, S, Totenhagen, JW, Hicks, CM, Erickson, RP, Trouard, TP & Trouard, TP 2008, 'MRI detects therapeutic effects in weanling Niemann-Pick type C mice', Journal of Neuroscience Research, vol. 86, no. 12, pp. 2802-2807. https://doi.org/10.1002/jnr.21707
Lope-Piedrafita, Silvia ; Totenhagen, John W. ; Hicks, Chad M. ; Erickson, Robert P. ; Trouard, Theodore P. ; Trouard, Theodore P. / MRI detects therapeutic effects in weanling Niemann-Pick type C mice. In: Journal of Neuroscience Research. 2008 ; Vol. 86, No. 12. pp. 2802-2807.
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