Chan W Y, Cushing W, Coffman M A, Rennert O M
Science. 1980 Apr 18;208(4441):299-300. doi: 10.1126/science.7367859.
Wilson's disease fibroblasts have an elevated intracellular copper concentration as compared to cultured control cells. A decreased ratio of copper to protein was observed in cytoplasmic protein (or proteins) having a molecular weight greater than or equal to 30,000 in Wilson's disease cells. The results of this culture study indicate its potential importance in the early unequivocal diagnosis of this disorder.
与培养的对照细胞相比,威尔逊氏病成纤维细胞的细胞内铜浓度升高。在威尔逊氏病细胞中,分子量大于或等于30,000的细胞质蛋白中观察到铜与蛋白的比例降低。这项培养研究的结果表明其在该疾病早期明确诊断中的潜在重要性。