肝豆状核变性培养细胞内铜分布的研究  

Distribution of copper in cultured skin fibroblasts of Wilson's disease

作　　者：陈嵘[1] 梁秀龄[1] 刘焯霖[1] 陈彪[1] 张影如[1] 

机构地区：[1]中山医科大学附属第一医院神经科

出　　处：《中华神经科杂志》1997年第1期12-15,共4页Chinese Journal of Neurology

基　　金：卫生部重点科研基金资助项目

摘　　要：通过确定肝豆状核变性培养细胞内铜在各亚细胞组分以及胞浆内不同分子量蛋白上的分布，以探讨本病的发病机理。方法测定肝豆状核变性患者、杂合子及对照组离体培养皮肤成纤维细胞的细胞浆、线粒体、溶酶体、微粒体和细胞浆的铜分布比例，并分析胞浆经ＳｅｐｈａｄｅｘＧ－７５凝胶层析后铜在不同分子量蛋白上的分布特点。结果２例患者分别有胞浆铜和溶酶体铜比例增高；患者胞浆内金属硫蛋白的含铜量明显高于对照组和杂合子。结论肝豆状核变性培养细胞内的铜异常沉积与金属硫蛋白密切相关，后者可能致本病的基本病理生理异常。Objective The study is to determine the copper distribution inside cultured skin fibroblasts of Wilson's disease. Method Copper distribution in subcellular fractions and in cytosol proteins with different molecular weights after sephadex G 75 chromatography in cultured skin fibroblasts of two patients with Wilson's disease and two heterozygotes along with three controls were meassured. Results The copper in cytosol and lysosomal fractions, and the copper combined in cytosol metallothionein were elevated in these two patients.Conclusion It was considered that the excessive deposition of copper in Wilson's disease was highly associated with metallothionein, which might play an essential role in the pathogenesis of the disease.

关 键 词：肝豆状核变性 铜代谢 细胞 

分 类 号：R742.402[医药卫生—神经病学与精神病学]