Epigenetic Regulation of ALS and CMT: A Lesson from Drosophila Models |
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Authors: | Masamitsu Yamaguchi Kentaro Omori Satoshi Asada Hideki Yoshida |
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Affiliation: | 1.Department of Applied Biology, Kyoto Institute of Technology, Matsugasaki, Sakyo-ku, Kyoto 606-8585, Japan; (K.O.); (S.A.);2.Kansai Gakken Laboratory, Kankyo Eisei Yakuhin Co. Ltd., Seika-cho, Kyoto 619-0237, Japan |
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Abstract: | Amyotrophic lateral sclerosis (ALS) is the third most common neurodegenerative disorder and is sometimes associated with frontotemporal dementia. Charcot–Marie–Tooth disease (CMT) is one of the most commonly inherited peripheral neuropathies causing the slow progression of sensory and distal muscle defects. Of note, the severity and progression of CMT symptoms markedly vary. The phenotypic heterogeneity of ALS and CMT suggests the existence of modifiers that determine disease characteristics. Epigenetic regulation of biological functions via gene expression without alterations in the DNA sequence may be an important factor. The methylation of DNA, noncoding RNA, and post-translational modification of histones are the major epigenetic mechanisms. Currently, Drosophila is emerging as a useful ALS and CMT model. In this review, we summarize recent studies linking ALS and CMT to epigenetic regulation with a strong emphasis on approaches using Drosophila models. |
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Keywords: | amyotrophic lateral sclerosis Charcot– Marie– Tooth disease epigenetics Drosophila melanogaster genetic screening long noncoding RNA histone modification enzyme |
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