Epigenetic Mechanisms of Neurodegeneration in Huntington's Disease

Title
Epigenetic Mechanisms of Neurodegeneration in Huntington's Disease
Authors
이정희황유진김기윤Neil W. Kowall류훈
Keywords
DNA methylation; Histone code; Chromatin remodeling; Noncoding RNA; microRNA; Huntington's disease; Therapeutics
Issue Date
2013-10
Publisher
Neurotherapeutics
Citation
VOL 10, NO 4, 664-676
Abstract
Huntington’s disease (HD) is an incurable and fatal hereditary neurodegenerative disorder of mid-life onset characterized by chorea, emotional distress, and progressive cognitive decline. HD is caused by an expansion of CAG repeats coding for glutamine (Q) in exon 1 of the huntingtin gene. Recent studies suggest that epigenetic modifications may play a key role in HD pathogenesis. Alterations of the epigenetic “histone code” lead to chromatin remodeling and deregulation of neuronal gene transcription that are prominently linked to HDpathogenesis. Furthermore, specific noncoding RNAs and microRNAs are associated with neuronal damage in HD. In this review, we discuss how DNA methylation, posttranslational modifications of histone, and noncoding RNA function are affected and involved in HD pathogenesis. In addition, we summarize the therapeutic effects of histone deacetylase inhibitors and DNA binding drugs on epigenetic modifications and neuropathological sequelae in HD. Our understanding of the role of these epigenetic mechanisms may lead to the identification of novel biological markers and new therapeutic targets to treat HD.
URI
http://pubs.kist.re.kr/handle/201004/46882
ISSN
19337213
Appears in Collections:
KIST Publication > ETC
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