威尔逊病中的细胞色素氧化酶缺乏症：一种推测的铜蓝蛋白功能。
Cytochrome oxidase deficiency in Wilson's disease: a suggested ceruloplasmin function.
作者信息
Shokeir M H, Shreffler D C
出版信息
Proc Natl Acad Sci U S A. 1969 Mar;62(3):867-72. doi: 10.1073/pnas.62.3.867.
Abstract
The hypothesis is advanced that ceruloplasmin functions in enzymatic transfer of copper to copper-containing enzymes, such as cytochrome oxidase. To test this hypothesis, leucocytes from Wilson's disease patients, heterozygous carriers, and normal subjects were assayed for cytochrome-oxidase activity. The data reported here show markedly reduced levels of activity in Wilson's disease cases and moderate reductions in heterozygous individuals relative to normal controls. These observations and a close correlation between the level of cytochrome-oxidase activity in the leucocytes and ceruloplasmin in the serum tend to support the hypothesis.
摘要
有人提出一种假说，即铜蓝蛋白在将铜酶促转移至含铜酶（如细胞色素氧化酶）的过程中发挥作用。为验证这一假说，对威尔逊氏病患者、杂合子携带者及正常受试者的白细胞进行了细胞色素氧化酶活性检测。此处报告的数据显示，威尔逊氏病患者的活性水平显著降低，杂合子个体相对于正常对照有中度降低。这些观察结果以及白细胞中细胞色素氧化酶活性水平与血清中铜蓝蛋白之间的密切相关性倾向于支持该假说。
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