期刊
EMBO REPORTS
卷 12, 期 7, 页码 735-742出版社
WILEY
DOI: 10.1038/embor.2011.86
关键词
MINT; myotonic dystrophy; SHARP; Spen; transcription
资金
- National Institutes of Health [R01 NS50861-04, R01 NS060839-03]
- Fondazione Telethon Funding Source: Custom
We describe a new mechanism by which CTG tract expansion affects myotonic dystrophy (DM1). Changes to the levels of a panel of RNAs involved in muscle development and function that are downregulated in DM1 are due to aberrant localization of the transcription factor SHARP (SMART/HDAC1-associated repressor protein). Mislocalization of SHARP in DM1 is consistent with increased CRM1-mediated export of SHARP to the cytoplasm. A direct link between CTG repeat expression and SHARP mislocalization is demonstrated as expression of expanded CTG repeats in normal cells recapitulates cytoplasmic SHARP localization. These results demonstrate a role for the inactivation of SHARP transcription in DM1 biology.
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