These compounds were investigated further to see whether they could target other pathogenic species with extended polyQ sequences, and 10O5 and AN2 were both found to be effective in degrading the mutant ataxin-3 (ATXN3) protein in fibroblasts derived from patients with the neurodegenerative disease spinocerebellar ataxia type 3 [111]. The gene discussed is ATXN3; the disease is Spinocerebellar ataxia type 3.