Article
Trehalose prevents the formation of aggregates of mutant ataxin-3 and reduces soluble ataxin-3 protein levels in an SCA3 cell model.
Neuroscience - 13 Sept 2024
Wang Zijian, Wang Min, Huang Yuhang, Ma Zhiwei, Gao Wenjing, Zhang Tian, Deng Jiexin, Cheng Xiaoxia, Liu Yingxun, Wang Bo, Qi Ying, Yang Min, He Fengqin
Abstract excerpt
Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder caused by mutant ataxin-3 with an abnormally expanded polyQ tract and is the most common dominantly inherited ataxia worldwide. There are no suitable therapeutic options for this disease. Autophagy, a defense mechanism against the toxic effects of aggregation-prone misfolded proteins, has been shown to have beneficial effects on...
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