Although the present observation is strikingly different than with other polyQ diseases, in which suppression of CREB-transcription is seen by nuclear localizations of mutated proteins [23], [24], [27], it should be noted that polyQ-aggregates are also seen in the cytoplasm in other polyQ diseases such as HD [28] and MJD [13], raising a hypothesis that CREB-transcriptional repression may be further enhanced by the formation of protein aggregates in the cytoplasm. This evidence concerns the gene CREB1 and Huntington disease.