Article
Reduction of toxic RNAs in myotonic dystrophies type 1 and type 2 by the RNA helicase p68/DDX5.
Proceedings of the National Academy of Sciences of the United States of America - 30 Jun 2015
Jones Karlie, Wei Christina, Schoser Benedikt, Meola Giovanni, Timchenko Nikolai, Timchenko Lubov
Abstract excerpt
Myotonic dystrophies type 1 (DM1) and type 2 (DM2) are neuromuscular diseases, caused by accumulation of CUG and CCUG RNAs in toxic aggregates. Here we report that the increased stability of the mutant RNAs in both types of DM is caused by deficiency of RNA helicase p68. We have identified p68 by studying CCUG-binding proteins associated with degradation of the mutant CCUG repeats. Protein levels of p68 are...
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