Epigenetic Mechanisms of Neurodegeneration in Huntington's Disease
- Epigenetic Mechanisms of Neurodegeneration in Huntington's Disease
- 이정희; 황유진; 김기윤; Neil W. Kowall; 류훈
- DNA methylation; Histone code; Chromatin
remodeling; Noncoding RNA; microRNA; Huntington's
- Issue Date
- VOL 10, NO 4, 664-676
- 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.
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- KIST Publication > ETC
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