【摘要】 通过比较不同浓度铜孵育后肝豆状核变性(HLD)患者皮肤成纤维细胞内铜、金属硫蛋白(MT)代谢的改变,发现经高浓度铜孵育后,患者胞浆内铜含量、MT含量及其铜结合量均明显增高,表明高铜孵育能促进本病遗传缺陷在皮肤成纤维细胞内进一步表达。我们认为,HLD的离体培养皮肤成纤维细胞模型是一种较为理想的研究模型。更多还原

【Abstract】 Copper and metallothionein metabolisms in cultured skin fibroblasts of Wilson’ s disease patients and controls were studied. It is found that when cultured in standard medium, the copper/protein ratio and metallothionein content in cytosol of patients showed no significant difference from controls. But after incubation in copper-rich medium, both copper/protein ratio and metallothinein content in patients were much higher than those of controls. The copper content binding in metallothionein in patients is higher than that in controls when cultured in standard medium, and is even higher when incubated in copper-rich medium. It is concluded that the cultured skin fibroblasts is an ideal model for study of Wilson’ s disease.更多还原