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探讨P型ATP酶阻滞剂、激动剂对肝豆状核变性患儿离体培养皮肤成纤维细胞胞浆铜含量的影响。方法采用高浓度铜及P型AFP酶阻滞剂（矾酸钠）、激动剂（长春新碱）孵育细胞，分析孵育6、12、24小时后，肝豆状核变性患儿、杂合子及正常儿童成纤维细胞胞浆中铜含量的变化。结果铜及长春新碱孵育后，患儿成纤维细胞胞浆钢水平在各时点均高于杂合子及正常儿童。铜及矾酸钠孵育后，患儿、杂合子及正常儿童胞浆铜含量在各时点均明显少于其他条件孵育后。结论患儿钢转运P型ATP酶对P型ATP酶阻滞剂的敏感性与杂合子及正常儿童相同，对激动剂的敏感性异常。P型ATP酶能减少患儿成纤维细胞对铜的摄取，在肝豆状核变性的治疗方面有一定的提示意义。
To investigate the effects of P-type ATPase blockers and agonists on cytosolic copper content in cultured skin fibroblasts from children with hepatolenticular degeneration. Methods High concentrations of copper and P-type AFP enzyme inhibitor (sodium vanadate) and agonist (vincristine) were used to incubate cells. After incubation for 6, 12 and 24 hours, the children with hepatolenticular degeneration and heterozygotes Changes of copper content in cytoplasm of normal children fibroblasts. Results After incubation with copper and vincristine, the levels of cytoplasmic fibroblasts in children were higher than those in heterozygous and normal children at all time points. After incubation with copper and sodium auric acid, the content of copper in cytoplasm of children, heterozygotes and normal children were obviously less than those of other conditions after incubation. Conclusions The sensitivity of P-type ATPase in steel transporters to P-type ATPase blockers is the same as that in heterozygous and normal children, and their sensitivity to agonists is abnormal. P-type ATPase can reduce the uptake of copper in children’s fibroblasts in the treatment of Wilson’s disease has some implications.