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Article

PIAS1 S510G variant acts as a genetic modifier of spinocerebellar ataxia type 3 by selectively impairing mutant ataxin-3 proteostasis.

The international journal of biochemistry & cell biology - 1 Nov 2024

Chang Yi-Ching, Tsai Yao-Chou, Chang En-Cheng, Hsu Yu-Chien, Huang Yi-Ru, Lee Yan-Hua, Tsai Yu-Shuen, Chen Yin-Quan, Lee Yi-Chung, Liao Yi-Chu, Kuo Jean-Cheng, Su Ming-Tsan, Yang Ueng-Cheng, Chern Yijuang, Cheng Tzu-Hao

Abstract excerpt

Dysregulated protein homeostasis, characterized by abnormal protein accumulation and aggregation, is a key contributor to the progression of neurodegenerative disorders such as Huntington's disease and spinocerebellar ataxia type 3 (SCA3). Previous studies have identified PIAS1 gene variants in patients with late-onset SCA3 and Huntington's disease. This study aims to elucidate the role of PIAS1 and its S510G...

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PMID: 39293559
DOI: 10.1016/j.biocel.2024.106662

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