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Semin Neurol 2011; 31(5): 470-483
DOI: 10.1055/s-0031-1299786
© Thieme Medical Publishers

Epigenetics in Nucleotide Repeat Expansion Disorders

Fang He1 , Peter K. Todd1
  • 1Department of Neurology, University of Michigan, Ann Arbor, Michigan
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Publikationsdatum:
21. Januar 2012 (online)

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ABSTRACT

Over the past 20 years, nucleotide repeat expansion disorders have informed our broader understanding of neurodevelopmental and neurodegenerative disease. This is especially true with regard to the contributions of epigenetic mechanisms to neurologic disease pathogenesis. In this review, the authors describe a few of the myriad ways in which epigenetic processes underlie aspects of repeat expansion disorder pathophysiology and discuss how therapies targeted at epigenetic modulation hold promise for many of these disorders.

KEYWORDS

Fragile X syndrome - myotonic dystrophy - spinocerebellar ataxia - Huntington's disease - neurodegeneration - HDAC inhibitors

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Peter K. ToddM.D. Ph.D. 

Assistant Professor of Neurology, Department of Neurology, University of Michigan

4001 BSRB, 109 Zina Pitcher Place, Ann Arbor, MI 48109

eMail: petertod@umich.edu