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Publication Detail

Title: RNA steady-state defects in myotonic dystrophy are linked to nuclear exclusion of SHARP.

Authors: Dansithong, Warunee; Jog, Sonali P; Paul, Sharan; Mohammadzadeh, Robabeh; Tring, Stephanie; Kwok, Yukwah; Fry, Rebecca C; Marjoram, Paul; Comai, Lucio; Reddy, Sita

Published In EMBO Rep, (2011 Jul 01)

Abstract: 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.

PubMed ID: 21637295 Exiting the NIEHS site

MeSH Terms: No MeSH terms associated with this publication

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