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

✍ Scribed by Silvia Lope-Piedrafita; John W. Totenhagen; Chad M. Hicks; Robert P. Erickson; Theodore P. Trouard

Publisher
John Wiley and Sons
Year
2008
Tongue
English
Weight
222 KB
Volume
86
Category
Article
ISSN
0360-4012

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✦ Synopsis

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 deficiencies in Npc1^–/–^ mice, consistent with the reduction in FA measured in 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.

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