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Author Notes:

Email Address: xli2@emory.edu

Conceived and designed the experiments: WJ SL XL.

Performed the experiments: WJ WW MG.

Analyzed the data: WJ WW.

Wrote the paper: WJ XL.

The authors have declared that no competing interests exist.

Subjects:

Research Funding:

This work was supported by grants from the National Institutes of Health (NS036232, NS041669 and AG019206).

Therapeutic Effect of Berberine on Huntington's Disease Transgenic Mouse Model.

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Journal Title:

PLoS ONE

Volume:

Volume 10, Number 7

Publisher:

, Pages e0134142-e0134142

Type of Work:

Article | Final Publisher PDF

Abstract:

Huntington disease (HD) represents a family of neurodegenerative diseases that are caused by misfolded proteins. The misfolded proteins accumulate in the affected brain regions in an age-dependent manner to cause late-onset neurodegeneration. Transgenic mouse models expressing the HD protein, huntingtin, have been widely used to identify therapeutics that may retard disease progression. Here we report that Berberine (BBR), an organic small molecule isolated from plants, has protective effects on transgenic HD (N171-82Q) mice. We found that BBR can reduce the accumulation of mutant huntingtin in cultured cells. More importantly, when given orally, BBR could effectively alleviate motor dysfunction and prolong the survival of transgenic N171-82Q HD mice. We found that BBR could promote the degradation of mutant huntingtin by enhancing autophagic function. Since BBR is an orally-taken drug that has been safely used to treat a number of diseases, our findings suggest that BBR can be tested on different HD animal models and HD patients to further evaluate its therapeutic effects.

Copyright information:

© 2015 Jiang et al.

This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits distribution of derivative works, making multiple copies, distribution, public display, and publicly performance, provided the original work is properly cited. This license requires credit be given to copyright holder and/or author, copyright and license notices be kept intact.

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