Our group previously provided evidence of an impairment of the autophagy pathway in a MJD rodent model and decreased levels of Beclin-1/ATG6, a component of the class III PI3 kinase complex required for autophagy initiation and autophagosome formation, in human fibroblasts from two MJD patients6. Here, BECN1 is linked to Spinocerebellar ataxia type 3.