铜孵育对肝豆状核变性离体培养皮肤成纤维细胞超微结构的影响

Microstructure of cultured skin fibroblasts of hepatolenticular degeneyation after copper incubation.

本实验研究不同浓度铜孵育、对肝豆状核变性(HLD)患者、杂合子离体培养皮肤成纤维细胞以观察其对超微结构的影响,初步探讨利用该模型研究HLD溶酶体缺陷的可能性,本实验结果提示HLD的溶酶体可能有摄铜的障碍或在HLD细胞的胞浆内可能存在与铜亲和力更高的化合物。

It has been suggested that Wilson's disease (hepatolenticular degeneration) might be caused by a lysosomal defect.In this article,the microstrure of cultured skin fibroblasts from the patients and heterozygotes with Wilson's disease after copper incubation was studied.It was suggested that there might be a defect of copper-intake in Wilson's disease lysosome,or an abnormal cytosol complex which had a higher copper affinity than lysosome, resulting in the copper deposition in cytosol of Wilson's disease cell.