Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice.
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ABSTRACT:
SUBMITTER: Di Pardo A
PROVIDER: S-EPMC3295265 | biostudies-other | 2012 Feb
REPOSITORIES: biostudies-other
Publications
Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice.
Di Pardo Alba A Maglione Vittorio V Alpaugh Melanie M Horkey Melanie M Atwal Randy S RS Sassone Jenny J Ciammola Andrea A Steffan Joan S JS Fouad Karim K Truant Ray R Sipione Simonetta S
Proceedings of the National Academy of Sciences of the United States of America 20120213 9
Huntington disease (HD) is a progressive neurodegenerative monogenic disorder caused by expansion of a polyglutamine stretch in the huntingtin (Htt) protein. Mutant huntingtin triggers neural dysfunction and death, mainly in the corpus striatum and cerebral cortex, resulting in pathognomonic motor symptoms, as well as cognitive and psychiatric decline. Currently, there is no effective treatment for HD. We report that intraventricular infusion of ganglioside GM1 induces phosphorylation of mutant ...[more]
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