MBNL Sequestration by Toxic RNAs and RNA Misprocessing in the Myotonic Dystrophy Brain
For some neurological disorders, disease is primarily RNA mediated due to expression of non-coding microsatellite expansion RNAs (RNAexp).Toxicity is thought to result from enhanced binding of proteins to these expansions and depletion from their normal cellular Grinders targets.However, experimental evidence for this sequestration model is lacking