Broad transcriptional dysregulation in HD was often linked to direct interaction of mHTT with proteins of the small interfering RNA (siRNA) machinery [22–27] and different transcriptional regulators such as CREB1, TBP, mSin3a, MBNL1, nucleolin, histone deacetylases (HDACs), or the DNA methyltransferase 1 (DNMT1). Here, MBNL1 is linked to Huntington disease.