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铜稳态的破坏是神经系统病变性疾病如Menkes综合征和Wilson’s症的发病基础。铜结合蛋白是指与铜具有高亲和力,在细胞中与铜的转运、分布及分泌过程密切相关的一系列铜受体和靶蛋白。本文主要从蛋白质结构和功能、铜结合位点、铜结合能力及结合铜后的胞内传递等方面对重要的几种铜结合蛋白进行综述,并探讨其在胞内铜稳态中的作用。
The failure of copper homeostasis is the basis for the pathogenesis of neurological disorders such as Menkes syndrome and Wilson’s disease. Copper-binding protein is a series of copper receptors and target proteins that have high affinity with copper and are closely related to copper transport, distribution and secretion in cells. This review summarizes several important copper-binding proteins in terms of protein structure and function, copper binding sites, copper binding capacity and intracellular delivery after binding to copper, and explores its role in intracellular copper homeostasis.