Additionally, though the symptoms of autosomal recessive spastic ataxia of the Charlevoix-Saguenay are mainly due to axonal deformities, the underlying mutation of the sacsin chaperone also results in neurofilament bundling in dendrites (Anderson et al., 2011; Gentil et al., 2019; Larivière et al., 2019). Here, SACS is linked to autosomal recessive spastic ataxia.