铜参与调控细胞能量代谢、线粒体呼吸和抗氧化等过程。细胞内铜代谢稳态依赖于铜的输入、存储和输出的动态平衡。铜代谢障碍(copper metabolic disorders)会影响细胞功能和生存。在多种神经退行性疾病和癌症中观察到铜含量增加,相关病理机制与铜超载引起的铜死亡(cuproptosis)密切相关。本文阐述和总结了细胞内铜代谢稳态维持机制、铜代谢障碍和铜死亡在神经退行性疾病和肿瘤发生中的作用及其涉及的分子机制。

Copper plays a crucial role in a variety of cellular processes, such as energy metabolism, mitochondrial respiration, and antioxidation. Intracellular copper homeostasis relies on the dynamic balance among copper intake, storage, and efflux. Disruptions in copper metabolism can impact cellular function and viability. Elevated copper levels have been observed in various neurodegenerative diseases and tumors, with the underlying pathological mechanisms closely associated with cuproptosis induced by copper overload. This article provides in-depth insights into intracellular copper homeostasis and its maintenance, as well as the roles and molecular mechanisms of copper metabolic disorders and cuproptosis in neurodegenerative diseases and tumorigenesis.