Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare early-onset neurodegenerative disease associated with mutations in the SACS gene, which encodes sacsin, a 520 kDa multidomain protein [1]. Here, SACS is linked to Autosomal recessive spastic ataxia of Charlevoix-Saguenay.