Wilson
發(fā)布時間：2018-05-02 08:51
本文選題：Wilson病 + 肝豆?fàn)詈俗冃?/strong>�。� 參考：《醫(yī)學(xué)研究雜志》2016年10期
【摘要】：金屬元素通過體內(nèi)平衡機(jī)制的調(diào)節(jié)和控制,使其保持正常水平和金屬內(nèi)穩(wěn)態(tài)(metal homeostasis,MH)。當(dāng)MH被破壞時,就會對機(jī)體造成各種病理性損害。各種金屬元素在機(jī)體內(nèi)的分布、含量和作用必須保持相對穩(wěn)定,不同的金屬元素之間存在著相互作用。Wilson病(Wilson's disease,WD)作為遺傳性銅代謝障礙疾病的代表,是少數(shù)幾個可以治療的神經(jīng)變性疾病,在其螯合療法中出現(xiàn)患者的癥狀惡化與金屬元素的重新分布和MH破壞有關(guān)。WD精準(zhǔn)驅(qū)銅目標(biāo)是在螫合療法中彈性調(diào)整螯合劑使用劑量,建立精準(zhǔn)的銅、鐵、鋅等金屬元素調(diào)控目標(biāo),減少繼發(fā)的金屬元素介導(dǎo)下蛋白質(zhì)聚集與氧化應(yīng)激的級聯(lián)反應(yīng),以恢復(fù)機(jī)體的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.
【作者單位】： 安徽中醫(yī)藥大學(xué)神經(jīng)病學(xué)研究所附屬醫(yī)院;
【基金】：國家臨床重點(diǎn)�？平ㄔO(shè)開放基金重點(diǎn)項(xiàng)目(2014lckf02004) 安徽省衛(wèi)生廳中醫(yī)藥科研基金資助項(xiàng)目(2012zy65)
【分類號】：R741
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本文編號：1833225
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