In this regard recent evidence shows that CGG trinucleotide repeats in permutation alleles of the fragile×gene (FMR1) cause neurodegeneration in Drosophila[36], [37] and involve disruption of RNA-binding protein function (hnRNP A2, Purα and CUG-BP1) as similarly described for alternative splicing regulators Muscleblind and CUG-BP1 in DM1. This evidence concerns the gene CELF1 and myotonic dystrophy type 1.