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RNA steady‐state defects in myotonic dystrophy are linked to nuclear exclusion of SHARP
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Titel: |
RNA steady‐state defects in myotonic dystrophy are linked to nuclear exclusion of SHARP |
In: | EMBO reports, 12, 2011, 7, S. 735-742 |
veröffentlicht: |
Springer Science and Business Media LLC
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Umfang: | 735-742 |
ISSN: |
1469-221X 1469-3178 |
DOI: | 10.1038/embor.2011.86 |
Zusammenfassung: | <jats:p>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.</jats:p> |
Format: | E-Article |
Quelle: | Springer Science and Business Media LLC (CrossRef) |
Sprache: | Englisch |