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Miglustat Reverts the Impairment of Synaptic Plasticity in a Mouse Model of NPC Disease
Joint Authors
Cardinale, Alessio
Merlo, Daniela
Tancredi, Virginia
Racaniello, Mauro
D’Arcangelo, G.
Grossi, D.
Zaratti, A.
Rufini, S.
Cutarelli, A.
Frank, Claudio
Source
Issue
Vol. 2016, Issue 2016 (31 Dec. 2016), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-01-14
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Abstract EN
Niemann-Pick type C disease is an autosomal recessive storage disorder, characterized by abnormal sequestration of unesterified cholesterol within the late endolysosomal compartment of cells and accumulation of gangliosides and other sphingolipids.
Progressive neurological deterioration and insurgence of symptoms like ataxia, seizure, and cognitive decline until severe dementia are pathognomonic features of the disease.
Here, we studied synaptic plasticity phenomena and evaluated ERKs activation in the hippocampus of BALB/c NPC1−/− mice, a well described animal model of the disease.
Our results demonstrated an impairment of both induction and maintenance of long term synaptic potentiation in NPC1−/− mouse slices, associated with the lack of ERKs phosphorylation.
We then investigated the effects of Miglustat, a recent approved drug for the treatment of NPCD.
We found that in vivo Miglustat administration in NPC1−/− mice was able to rescue synaptic plasticity deficits, to restore ERKs activation and to counteract hyperexcitability.
Overall, these data indicate that Miglustat may be effective for treating the neurological deficits associated with NPCD, such as seizures and dementia.
American Psychological Association (APA)
D’Arcangelo, G.& Grossi, D.& Racaniello, Mauro& Cardinale, Alessio& Zaratti, A.& Rufini, S.…[et al.]. 2016. Miglustat Reverts the Impairment of Synaptic Plasticity in a Mouse Model of NPC Disease. Neural Plasticity،Vol. 2016, no. 2016, pp.1-9.
https://search.emarefa.net/detail/BIM-1113109
Modern Language Association (MLA)
D’Arcangelo, G.…[et al.]. Miglustat Reverts the Impairment of Synaptic Plasticity in a Mouse Model of NPC Disease. Neural Plasticity No. 2016 (2016), pp.1-9.
https://search.emarefa.net/detail/BIM-1113109
American Medical Association (AMA)
D’Arcangelo, G.& Grossi, D.& Racaniello, Mauro& Cardinale, Alessio& Zaratti, A.& Rufini, S.…[et al.]. Miglustat Reverts the Impairment of Synaptic Plasticity in a Mouse Model of NPC Disease. Neural Plasticity. 2016. Vol. 2016, no. 2016, pp.1-9.
https://search.emarefa.net/detail/BIM-1113109
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1113109