铜稳态失调在肌萎缩侧索硬化发病机制中的研究进展
Research advances in copper homeostasis disorder in the pathogenesis of amyotrophic lateral sclerosis
张莹 1色瑶 1姜宏佺1
作者信息
- 1. 哈尔滨医科大学附属第一医院神经内科,黑龙江 哈尔滨 150001
- 折叠
摘要
铜对维持细胞的正常代谢功能至关重要,参与细胞中多种生理过程,包括细胞呼吸、神经肽加工和铁运输等,维持铜稳态具有重要意义.在中枢神经系统中,铜稳态参与突触功能调节及髓鞘形成过程等,铜稳态失调与多种神经退行性疾病的发生密切相关,近年研究表明,铜转运ATP酶α(ATP7A)突变后导致的铜稳态失调,可能导致了肌萎缩侧索硬化(ALS)的发生发展.本文通过综述铜稳态失调在ALS发病机制的研究进展,提出了维持铜稳态可能为ALS治疗提供新靶点的展望.
Abstract
Copper plays an important role in maintaining the normal metabolic function of cells and participates in a variety of physiological processes in cells,including cell respiration,neuropeptide processing,and iron transport,and therefore,it is of great significance to maintain copper homeostasis.In the central nervous system,copper homeostasis is involved in the regulation of synaptic function and the formation of myelin sheath,and copper homeostasis disorder is closely associated with the development of various neurodegenerative diseases.Recent studies have shown that copper ho-meostasis disorder caused by the mutation of ATPase copper transporting α may lead to the development and progression of amyotrophic lateral sclerosis(ALS).This article reviews the research advances in copper homeostasis disorder in the pathogenesis of ALS and points out that maintaining copper homeostasis may provide a new target for the treatment of ALS.
关键词
肌萎缩侧索硬化/铜稳态/铜转运ATP酶αKey words
Amyotrophic lateral sclerosis/Copper homeostasis/ATPase copper transporting α引用本文复制引用
基金项目
黑龙江省自然科学基金优秀青年项目(YQ2020H013)
国家自然科学基金青年科学基金(81601102)
黑龙江省博士后启动基金(LBH-Q19128)
中国初级卫生保健基金会(2022HX034)
出版年
2024