Abstract #2093
Partial preservation of white matter in a murine model of Niemann-Pick type C disease with therapeutic intervention: An ex vivo DTI study
Min-Hui Cui 1,2 , Cristin D. Davidson 3 , Ziqin Yuan 1 , Kwame Kyei 1 , Steven U. Walkley 3 , and Craig A. Branch 1,2
1
Gruss Magnetic Resonance Research Center,
Albert Einstein College of Medicine, Bronx, New York,
United States,
2
Radiology,
Albert Einstein College of Medicine, Bronx, New York,
United States,
3
Neuroscience,
Albert Einstein College of Medicine, Bronx, New York,
United States
A mouse model of Niemann-Pick type C disease, a fatal
inherited metabolic disorder, shows disease amelioration
upon administration of miglustat, cyclodextrin, or a
combination of both compounds. Here, we show
preservation of white matter as evidenced by increased
FA in two distinct brain regions, the corpus callosum
and fimbria. Cyclodextrin and combination therapy
provided greater benefit than miglustat alone, following
suit with what we have observed in longevity and in
reductions of stored cholesterol and glycosphingolipids
within treated NPC1-/- mice.
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