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Epigenetic Mechanisms Involved in Huntington's Disease Pathogenesis

Article type: Review Article

Authors: Glajch, Kelly E. | Sadri-Vakili, Ghazaleh

Affiliations: MassGeneral Institute for Neurodegenerative Disease, Massachusetts General Hospital, Boston, MA, USA

Note: [] Correspondence to: Ghazaleh Sadri-Vakili, Ph.D., MassGeneral Institute for Neurodegenerative Disease, Massachusetts General Hospital, 114 16th Street, Charlestown, MA 02129-4404, USA. Tel.: +1 617 724 1487; Fax: +1 617 724 1480; E-mail: gsadrivakili@mgh.harvard.edu

Keywords: Epigenetics, Huntington's disease, DNA methylation, histone modifications, huntingtin, transcription

DOI: 10.3233/JHD-140134

Journal: Journal of Huntington's Disease, vol. 4, no. 1, pp. 1-15, 2015

Published: 2015
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Abstract

Transcriptional dysregulation is an early event and may be an important pathological mechanism in Huntington's disease (HD). However, the exact process that leads to alterations in gene expression in HD is not clear. One potential mechanism underlying transcriptional abnormalities in HD may be epigenetic alterations which regulate gene expression without changing the DNA sequence. Previous work has demonstrated that epigenetic marks, such as DNA methylation and post-translational modifications of histone proteins, are significantly altered in HD cellular and animal models as well as HD patients. Furthermore, studies have shown a therapeutic role for histone deacetylase (HDAC) inhibitors in numerous HD models. Here, we review a range of studies describing epigenetic changes in HD as well as several potential therapeutics that target aberrant epigenetic alterations in HD.

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