[HTML][HTML] RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1

NA Timchenko, ZJ Cai, AL Welm, S Reddy… - Journal of Biological …, 2001 - ASBMB
An RNA CUG triplet repeat binding protein, CUGBP1, regulates splicing and translation of
various RNAs. Expansion of RNA CUG repeats in the 3′-untranslated repeat of the mutant
myotonin protein kinase (DMPK) mRNA in myotonic dystrophy (DM) is associated with
alterations in binding activity of CUGBP1. To investigate whether CUGBP1 is directly
affected by expansion of CUG repeats in DM tissues, we examined the intracellular status of
CUGBP1 in DM patients as well as in cultured cells over expressing RNA CUG repeats. The …