Wilson

发布时间：2018-05-02 08:51

  本文选题：Wilson病 + 肝豆状核变性　； 参考：《医学研究杂志》2016年10期

【摘要】：金属元素通过体内平衡机制的调节和控制,使其保持正常水平和金属内稳态(metal homeostasis,MH)。当MH被破坏时,就会对机体造成各种病理性损害。各种金属元素在机体内的分布、含量和作用必须保持相对稳定,不同的金属元素之间存在着相互作用。Wilson病(Wilson's disease,WD)作为遗传性铜代谢障碍疾病的代表,是少数几个可以治疗的神经变性疾病,在其螯合疗法中出现患者的症状恶化与金属元素的重新分布和MH破坏有关。WD精准驱铜目标是在螫合疗法中弹性调整螯合剂使用剂量,建立精准的铜、铁、锌等金属元素调控目标,减少继发的金属元素介导下蛋白质聚集与氧化应激的级联反应,以恢复机体的MH。
[Abstract]:Metal elements maintain normal level and metal homeostasisof MHH by adjusting and controlling in vivo equilibrium mechanism. When MH is destroyed, it causes various pathological damage to the body. The distribution, content and action of various metal elements in the body must be relatively stable. There is interaction between different metal elements. Wilson's disease Wilsonsdisease (WD) is the representative of hereditary copper metabolic disorders. This is one of the few treatable neurodegenerative diseases in which symptoms deteriorate in patients and are related to the redistribution of metal elements and the destruction of MH. The precise copper remover is targeted at elastically adjusting the dosage of chelating agents in chelating therapy. In order to restore MHs, precise targets of copper, iron, zinc and other metal elements were established to reduce the cascade reaction between protein aggregation and oxidative stress mediated by secondary metal elements.
【作者单位】： 安徽中医药大学神经病学研究所附属医院;
【基金】：国家临床重点专科建设开放基金重点项目(2014lckf02004) 安徽省卫生厅中医药科研基金资助项目(2012zy65)
【分类号】：R741
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本文编号：1833225