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近年来,大量的研究表明,自由基参与许多疾病的病理生理过程。肝豆状核变性(HLD)患者血浆铜蓝蛋白(CP)低下为其特征性生化表现,体内二价铁氧化为三价铁需在CP的酶促作用下自由基才得以迅速清除,由此推想HLD患者有可能存在氧自由基的慢性损害。近年的研究认为组织及血液中α_2-巨球蛋白(α_2-M)不仅为一种血浆蛋白酶抑制剂,而且具有清除自由基的作用。本文同时测定HLD患者血浆α_2-M含量及红细胞超氧化物歧化酶(SOD)的活力,以探讨其在本病发病过程中的意义。
In recent years, a large number of studies have shown that free radicals are involved in the pathophysiology of many diseases. The decrease of ceruloplasmin (CP) in patients with hepatolenticular degeneration (HLD) is the characteristic biochemical manifestation. The oxidation of ferrous iron to ferric iron in vivo needs the rapid elimination of free radicals by the enzymatic action of CP, It is hypothesized that HLD patients may have chronic damage of oxygen free radicals. In recent years, studies suggest that α 2 -macroglobulin (α 2 -M) in tissues and blood is not only a plasma protease inhibitor, but also has the function of eliminating free radicals. In this paper, the determination of α2-M in plasma and the activity of erythrocyte superoxide dismutase (SOD) in HLD patients at the same time to investigate its significance in the pathogenesis of this disease.