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MRI detects therapeutic effects in weanling Niemann‐Pick type C mice
Author(s) -
LopePiedrafita Silvia,
Totenhagen John W.,
Hicks Chad M.,
Erickson Robert P.,
Trouard Theodore P.
Publication year - 2008
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.21707
Subject(s) - niemann–pick disease, type c , fractional anisotropy , white matter , diffusion mri , in vivo , myelin , medicine , endocrinology , pathology , chemistry , biology , magnetic resonance imaging , disease , central nervous system , microbiology and biotechnology , radiology
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.