Epigenetic profiles in polyglutamine disorders

Abstract

The dominant polyglutamine (polyQ) disorders are a group of progressive and incurable neurodegenerative disorders, which are caused by unstable expanded CAG trinucleotide repeats in the coding regions of their respective causative genes. The most prevalent polyQ disorders worldwide are Huntington’s disease and spinocerebellar ataxia type 3. Epigenetic mechanisms, such as DNA methylation, histone modifications and chromatin remodeling and noncoding RNA regulation, regulate gene expression or genome function. Epigenetic dysregulation has been suggested to play a pivotal role in the pathogenesis of polyQ disorders. Here, we summarize the current knowledge of epigenetic changes present in several representative polyQ disorders and discuss the potentiality of miRNAs as therapeutic targets for the clinic therapy of these disorders.

Keywords::

• epigenetic dysregulation
• pathogenesis
• polyglutamine disorders

Financial&competing interests disclosure

This work was supported by NSFC (91754204, 31300880), National Key Research and Development Program of China 2017YFC1001001, NSFC81630078, CAS Strategic Priority Research Program XDB14030300, and the State Key Laboratory of Membrane Biology. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Additional information

Funding

This work was supported by NSFC (91754204, 31300880), National Key Research and Development Program of China 2017YFC1001001, NSFC81630078, CAS Strategic Priority Research Program XDB14030300, and the State Key Laboratory of Membrane Biology. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. No writing assistance was utilized in the production of this manuscript.