In this video we interviewed Aliza Ben-Varon, a former Graduate Student working with Dr Jeff Carroll at the University of Washington. This research group has done a fantastic job in characterizing a mouse model that mimics the human form of DRPLA. Using genetic engineering, scientists inserted the human ATN1 gene into mice with 112 CAG repeats so that the mice would develop DRPLA. ASOs are being tested in these DRPLA mice to determine if they can slow down or reverse DRPLA progression.
You can watch the interview here and you can read more about this mouse model and their research here.