Kayser-Fleischer环与肝豆状核变性患者细胞内铜及金属硫蛋白代谢的关系  

Metablism of copper and metallothionein in Wilson's disease

作　　者：陈慧敏[1] 余涵[1] 余腾[2] 尹景岗[2] 杨尊之[2] 毕毅敏[1] 张向东[2] 

机构地区：[1]新乡医学院三附院,453003 [2]新乡医学院眼科医院

出　　处：《眼科新进展》1997年第4期206-208,共3页Recent Advances in Ophthalmology

摘　　要：目的检测K－F（Kayser－Fleilcher）环阳性及阴性患者细胞内铜及金属硫蛋白（Metallithionern，MT）代谢情况，探讨K－F环形成的细胞生物化学基础。方法采用离体培养皮肤成纤维细胞模型，用高钢（300μmol·L－1）孵育促使铜及MT代谢的遗传缺陷在细胞内的表达。比较孵育不同时点各组间细胞内铜及MT代谢的差异。结果高铜孵育后患者细胞内铜及MT水平均较对照组为高，且K－F环阳性患者与K－F环阴性患者间也有显著差异（P＜0．05）。结论K－F环与患者细胞内铜及MT代谢的遗传缺陷及表达水平有关。Objectives: Examining Levels of cellular copper andmetallothionein (MT ) in wilson's disease patients with andwithout K-F ring, to research cellular biochemistric mechanism of K-F ring.Methods: Copper and MT metablism were studied incuttured skin fibroblasts of patients and controls, Cells wereincubated in standard medium and medium with copper(300μmll·L-1)for 12、 24 and 36 hours. The cytosolic copper/protein ratio and cytosolic MT were compared betweengroups and at incubated periods res pectively.Results: After incubation of copperrich medium for 12hours, cytosolic copper/protein ratio in patients was higherthan that in controls, the levels of cytosolic MT were obviously higher in K-F negative patients than that in positivepatients and controls. and incubated for 24 and 36 hours theratios in K-F ring peitive patients wer higher than that innegative petients and controls.Conclusion: K-F ring is not only a sign of eye in wilson's disease but may be a metabolic sign of cellular copperand MT in Patients.

关 键 词：铜 金属硫蛋白 肝豆状核变性 K-F环 角膜 

分 类 号：R742.4[医药卫生—神经病学与精神病学] R771.3[医药卫生—临床医学]