Our data showing alterations in mitochondria and ER distribution in distal dendrites in Sacs–/– primary PCs phenocopy plectin or myosin Va deficiency in neurons, supporting the potential involvement of these 2 proteins in ARSACS pathogenesis (24, 26). Here, MYO5A is linked to Autosomal recessive spastic ataxia of Charlevoix-Saguenay.