Epigenetic profiles in polyglutamine disorders

• Published in: Epigenomics Vol. 10; no. 1; pp. 9 - 25
• Main Authors: Liu, Hongmei, Tang, Tie-Shan, Guo, Caixia
• Format: Journal Article
• Language: English
• Published: England Future Medicine Ltd 01.01.2018
• Subjects: Ataxia; Atrophy; Cell cycle; Chromatin remodeling; Dementia; Deoxyribonucleic acid; Disease; Disorders; DNA; DNA methylation; Dysphagia; epigenetic dysregulation; Epigenetics; Gait; Gene expression; Genomes; Genomics; Machado-Joseph disease; MicroRNAs; Neurodegenerative diseases; Non-coding RNA; Pathogenesis; Polyglutamine; polyglutamine disorders; Proteins; Restless legs syndrome; Ribonucleic acid; RNA; Spinal cord; Therapeutic applications; Trinucleotide repeat diseases; Trinucleotide repeats; epigenetic dysregulation; pathogenesis; polyglutamine disorders
• ISSN: 1750-1911; 1750-192X
• DOI: 10.2217/epi-2017-0089

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.