Pathogenic mutations in several components of the HOPS complex, including VPS11, VPS16, and VPS41 that is known to functionally interact with BORC and is involved in autophagosome-lysosome fusion and late-endosome to lysosome maturation,15,16 have been described to be causative for cases with movement disorders like ataxia and dystonia.3,43–47 Thus, lysosomal dysfunction underlying these genetic defects might be a unifying factor in the pathogenesis of these movement disorders. This evidence concerns the gene VPS41 and Ataxia.