This project is jointly funded by the Ataxia Charlevoix- Saguenay Foundation and the Massachusetts General ARSACS Fundraiser, fund created by families in the USA. Recent studies and clinical trials have demonstrated that omaveloxolone (brand name Skyclarys) can improve clinical symptoms in Friedreich’s ataxia. Drs. Schmahmann and Lin from Harvard University will administer Skyclarys to a mouse model of ARSACS and investigate whether Skyclarys can change the disease course in these mice. We believe the findings of the proposed study will be critical in determining whether omaveloxolone is appropriate for clinical trials in ARSACS.