Huntington’s disease (HD) is caused by expansion of polyglutamine repeats in the protein huntingtin, which affects the corpus striatum of the brain. The polyglutamine repeats in mutant huntingtin cause its aggregation and elicit toxicity by affecting several cellular processes, which include dysregulated organellar stress responses. The Golgi apparatus not only plays key roles in the transport, processing, and targeting of proteins, but also functions as a sensor of stress, signaling through the Golgi stress response. Unlike the endoplasmic reticulum (ER) stress response, the Golgi stress response is relatively unexplored. This review focuses on the molecular mechanisms underlying the Golgi stress response and its intersection with cysteine metabolism in HD.

中文翻译：

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亨廷顿病中高尔基体应激反应与半胱氨酸代谢的信号重叠

亨廷顿舞蹈病 (HD) 是由亨廷顿蛋白中聚谷氨酰胺重复序列的扩增引起的，它会影响大脑的纹状体。突变体亨廷顿蛋白中的聚谷氨酰胺重复通过影响多种细胞过程（包括失调的细胞器应激反应）引起其聚集并引起毒性。高尔基体不仅在蛋白质的运输、加工和靶向中起着关键作用，而且还充当压力传感器，通过高尔基体压力反应发出信号。与内质网 (ER) 应激反应不同，高尔基体应激反应相对未知。本综述侧重于高尔基体应激反应的分子机制及其与 HD 中半胱氨酸代谢的交叉。