P型ATP酶阻滞剂和激动剂对肝豆状核变性成纤维细胞胞浆铜的影响

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。

Influence of antagonist and agonist of P-type ATPase on Cytoplasmic copper of fibroblasts in Wilson disease

To research the influenee of antagonist and agonist of P-typeATPase on cytoplasmic copper of cultured skin fibroblasts in Wilson disease invitro.Methods Incubations of fibroblasts were achieved with copper and P type ATPare antagnist (Vanadate), agonist (vincristine) for 6, 12 and 24hours. Cytoplasmic copper concentrations in fibroblasts were compared amongWilson disease children, heterozygotes and controls.Results Cytoplasmic copper levels in fibroblasts of Wilson disease children were higher than that in heterozygotes and coneals after incubalion withcopper and copper with vincristine. Cytoplasnic copper concenbetions of threecoups incubated with vanadate were lower than that with other agents, whilebeing on about the same level between groups.Conclusion P- type ATPase of Wilson disease children might be insensitive to vincristine but remain sensitive to vanadate. Vanadate would reducethe cellular coppar uptake, and it has significance to treat the Wilson disease.