Multimodal characterization of sensorimotor peripheral neuropathy in a preclinical mouse model of SCA3

Spinocerebellar ataxia type 3 (SCA3), also known as Machado Joseph disease, is the most common dominantly inherited ataxia caused by a toxic gain-of-function CAG expansion in ATXN3. Currently, no effective preventive or disease-modifying treatments exist for this uniformly fatal disorder. This progressive, multisystem disorder is typified by neuronal degeneration and demyelination of critical...