A marked paucity of granule cells in the developing cerebellum of the Npc1−/− mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrin

@inproceedings{Nusca2014AMP,
  title={A marked paucity of granule cells in the developing cerebellum of the Npc1−/− mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrin},
  author={Stefania Nusca and Sonia Canterini and Giampiero Palladino and Francesco Bruno and Feliciana Mangia and Robert P. Erickson and Maria Teresa Fiorenza},
  booktitle={Neurobiology of Disease},
  year={2014}
}
In this study we show that postnatal development of cerebellar granule neurons (GNs) is defective in Npc1(-/-) mice. Compared to age-matched wild-type littermates, there is an accelerated disappearance of the external granule layer (EGL) in these mice. This is due to a premature exit from the cell cycle of GN precursors residing at the level of the EGL. As a consequence, the size of cerebellar lobules of these mice displays a 20%-25% reduction compared to that of age-matched wild-type mice… CONTINUE READING
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117–126 Niemann–Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression

  • S. Nusca
  • Neurobiology of Disease
  • 2014

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