Provided are compounds, methods, and pharmaceutical compositions for reducing the amount or activity of ATXN3 mRNA in a cell or animal, and in certain instances reducing the amount of Ataxin-3 protein in a cell or animal. Such compounds, methods, and pharmaceutical compositions are useful to prevent or ameliorate at least one symptom or hallmark of a neurodegenerative disease. Such symptoms and hallmarks include ataxia, neuropathy, and aggregate formation. Such neurodegenerative diseases include SCA3.本發明提供用於減少細胞或動物中之ATXN3 mRNA之量或活性及在某些情況下減少細胞或動物中之Ataxin-3蛋白之量的化合物、方法及醫藥組合物。此種化合物、方法及醫藥組合物適用於預防或改善神經退化性疾病之至少一種症狀或標誌。此種症狀及標誌包括運動失調、神經病變及聚集體形成。此種神經退化性疾病包括SCA3。