METABOLISM OF COPPER IN WILSONS DISEASE AND IN NORMAL SUBJECTS - STUDIES WITH CU-64
METABOLISM OF COPPER IN WILSONS DISEASE AND IN NORMAL SUBJECTS - STUDIES WITH CU-64
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DOI：
10.1016/0002-9343(54)90258-2
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发表时间：
1954-01-01
影响因子：
5.9
通讯作者：
SELVERSTONE, B
中科院分区：
文献类型：
--
作者：
EARL, CJ;MOULTON, MJ;SELVERSTONE, B
1.1.已将标记的氯化铜 (Cu-64Cl2) 溶液口服给予 5 名威尔逊氏病患者和 4 名正常受试者，并研究了血浆和尿液中的 Cu-64 活性。对两名正常受试者和一名威尔逊氏病患者静脉注射 Cu-64Cl2 后进行了类似的研究。2.2．两组的血浆 Cu-64 活性曲线惊人地相似。然而，威尔逊氏病患者的 Cu-64 尿液“清除率”远高于正常受试者。3.3．血浆蛋白的简单分离提供了额外的证据，表明威尔逊氏病的特定缺陷是铜蓝蛋白合成失败。在此基础上提出了一个假设，可以解释该疾病的相关生化异常。4.4．在体外，通过超滤添加了Cu-64Cl2的血清可以证明少量可扩散的标记铜。
1.1. A solution of labelled cupric chloride (Cu-64Cl2) has been administered orally to five patients with Wilson's disease and to four normal subjects, and the Cu-64 activity of the plasma and urine has been studied. A similar investigation has been carried out after intravenous administration of Cu-64Cl2to two normal subjects and to one patient with Wilson's disease.2.2. Curves of plasma Cu-64 activity were surprisingly similar in the two groups. Urinary "clearance" of Cu-64 was, however, much greater in patients with Wilson's disease than in normal subjects.3.3. Simple fractionation of the plasma proteins provides additional evidence that the specific defect in Wilson's disease is a failure of synthesis of caeruloplasmin. A hypothesis is presented by which the associated biochemical abnormalities of the disease may be explained on this basis.4.4. In vitro, a small amount of diffusible labelled copper can be demonstrated by ultrafiltration of serum to which Cu-64Cl2has been added.