期刊
ANTIOXIDANTS
卷 10, 期 9, 页码 -出版社
MDPI
DOI: 10.3390/antiox10091468
关键词
Golgi apparatus; Huntington's disease; cysteine; transsulfuration; Golgi stress response; integrated stress response
资金
- American Heart Association (AHA)/Paul Allen Frontiers Group [19PABH134580006]
- National Institutes of Health, NIA [1R21AG07368401]
Huntington's disease is caused by polyglutamine repeats in the huntingtin protein, leading to toxicity by affecting cellular processes, including dysregulated organellar stress responses. The Golgi apparatus, functioning as a stress sensor, plays a crucial role in protein transport and processing, but its stress response remains largely unexplored. This review focuses on the molecular mechanisms underlying the Golgi stress response and its interaction with cysteine metabolism in the context of Huntington's disease.
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.
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