Article
n-Butylidenephthalide Modulates Autophagy to Ameliorate Neuropathological Progress of Spinocerebellar Ataxia Type 3 through mTOR Pathway.
International journal of molecular sciences - 13 Jun 2021
Lee Jui-Hao, Lin Si-Yin, Liu Jen-Wei, Lin Shinn-Zong, Harn Horng-Jyh, Chiou Tzyy-Wen
Abstract excerpt
Spinocerebellar ataxia type 3 (SCA3), a hereditary and lethal neurodegenerative disease, is attributed to the abnormal accumulation of undegradable polyglutamine (polyQ), which is encoded by mutated ataxin-3 gene (ATXN3). The toxic fragments processed from mutant ATXN3 can induce neuronal death, leading to the muscular incoordination of the human body. Some treatment strategies of SCA3 are preferentially focused...
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