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Neural Plasticity
Volume 2016 (2016), Article ID 3830424, 9 pages
http://dx.doi.org/10.1155/2016/3830424
Research Article

Miglustat Reverts the Impairment of Synaptic Plasticity in a Mouse Model of NPC Disease

1Department of Medical System, University of Rome Tor Vergata, 00133 Rome, Italy
2Department of Cell Biology and Neuroscience, Istituto Superiore di Sanità, 00161 Rome, Italy
3IRCCS San Raffaele Pisana, 00163 Rome, Italy
4Department of Biology, University of Rome Tor Vergata, 00133 Rome, Italy
5National Centre for Rare Diseases, Istituto Superiore di Sanità, 00161 Rome, Italy

Received 24 July 2015; Revised 15 October 2015; Accepted 1 November 2015

Academic Editor: Lucas Pozzo-Miller

Copyright © 2016 G. D’Arcangelo et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Supplementary Material

The video shows the different behaviours observed in NPC1−/− not treated and in NPC1−/− treated mice with Miglustat for 20 days. The treated animal does not show insurgence of neurological symptoms presenting a normal function motor system while the NPC1−/− not treated mouse does not move quickly to explore the cage, often it remains in the same position displaying ataxia and tremor.

  1. Supplementary Material