SACS knockout mice, investigated by Larivière (2015), revealed typical ARSACS symptoms, loss of Purkinje cells in the cerebellum and abnormal aggregation on non-phosphorylated neurofilaments in the somatodendritic brain region with altered mitochondrial dynamics [12]. This evidence concerns the gene SACS and Autosomal recessive spastic ataxia of Charlevoix-Saguenay.