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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。
In this study, different concentrations of copper incubated in patients with hepatolenticular degeneration (HLD), heterozygous cultured skin fibroblasts in vitro to observe its effects on ultrastructure, preliminary study of the use of the model of HLD lysosomal defects Possibilities, the results of this experiment suggest that lysosomes of HLD may have copper-induced obstacles or compounds with higher copper affinity may exist in the cytoplasm of HLD cells.