Subcellular, biochemical and biophysical alterations in two glial cell models of ARSACS
Fernanda Murtinheira,
Ana Sofia Boasinha,
João Belo
et al.
Abstract:Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a developmental and degenerative disorder caused by loss-of-function mutations in the gene that codifies for the sacsin chaperone. Sacsin was initially described as a neuronal protein but is found in various cell types, including astroglial, microglial, kidney, and skin cell lines. We and others have shown that virtually all cell and animal models of ARSACS show disruption of intermediate filament (IF) cytoskeleton and organelle distribution… Show more
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