Article
Small Molecules Inducing Autophagic Degradation of Expanded Polyglutamine Protein through Interaction with Both Mutant ATXN3 and LC3.
International journal of molecular sciences - 4 Oct 2024
Lin Te-Hsien, Chen Wan-Ling, Hsu Shao-Fan, Chen I-Cheng, Lin Chih-Hsin, Chang Kuo-Hsuan, Wu Yih-Ru, Chen Yi-Ru, Yao Ching-Fa, Lin Wenwei, Lee-Chen Guey-Jen, Chen Chiung-Mei
Abstract excerpt
Polyglutamine (polyQ)-mediated spinocerebellar ataxia (SCA), including SCA1, 2, 3, 6, 7, and 17, are caused by mutant genes with expanded CAG repeats, leading to the intracellular accumulation of aggregated proteins, the production of reactive oxygen species, and cell death. Among SCA, SCA3 is caused by a mutation in the ATXN3 (ataxin-3) gene. In a circumstance of polyQ aggregation, the autophagic pathway is...
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