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Permanent link (DOI): https://doi.org/10.7939/R3DS3N

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Lipid dysfunction in Huntington Disease -"Molecular Mechanisms and Therapy" Open Access

Descriptions

Other title
Subject/Keyword
Lipids
Huntington Disease
Type of item
Thesis
Degree grantor
University of Alberta
Author or creator
Di Pardo, Alba
Supervisor and department
Sipione, Simonetta (Pharmacology)
Examining committee member and department
Fouad, Karim (Centre for Neuroscience)
Posse De Chaves, Elena (Pharmacology)
Department
Department of Pharmacology
Specialization

Date accepted
2013-01-08T11:15:32Z
Graduation date
2013-06
Degree
Master of Science
Degree level
Master's
Abstract
Huntington disease (HD) is a neurodegenerative disorder characterized by motor and cognitive symptoms. In HD patients, the protein huntingtin contains an abnormal expansion of a polyglutamine tract, which leads to the selective dysfunction and death of striatal and cortical neurons. Among other cellular dysfunctions, cholesterol and ganglioside GM1 synthesis are affected in HD neurons. In this thesis I demonstrated that impaired cholesterol metabolism in HD cells results from aberrant interaction of mutant huntingtin with the transcription factor Sterol Regulatory Element-Binding Protein 2 (SREBP2). I also showed that administration of GM1 restores normal motor behavior in HD mice.My studies have led to a better understanding of the causes of cholesterol metabolism dysregulation in HD, and have identified GM1 as a potential therapy for the disease
Language
English
DOI
doi:10.7939/R3DS3N
Rights
Permission is hereby granted to the University of Alberta Libraries to reproduce single copies of this thesis and to lend or sell such copies for private, scholarly or scientific research purposes only. Where the thesis is converted to, or otherwise made available in digital form, the University of Alberta will advise potential users of the thesis of these terms. The author reserves all other publication and other rights in association with the copyright in the thesis and, except as herein before provided, neither the thesis nor any substantial portion thereof may be printed or otherwise reproduced in any material form whatsoever without the author's prior written permission.
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