Numerous neurodegenerative diseases have been reported to develop neuropathology, including protein aggregate formation within neurons, particularly including aggregates of mutant protein species, such as mutated TDP-43 in amyotrophic lateral sclerosis, amyloid-b in Alzheimer’s disease, a-synuclein in Parkinson’s disease, huntingtin in Huntington’s disease, and ataxin-3 in Spinocerebellar ataxia 3 [1,2,3,4,5]. The gene discussed is ATXN3; the disease is Machado-Joseph disease.