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遗传性 C2 缺乏症:缺乏免疫化学检测到的血清中 C2 蛋白的个体。

Inherited c'2 deficiency in man: lack of immunochemically detectable c'2 protein in serums from deficient individuals.

出版信息

Science. 1968 Sep 13;161(3846):1149-51. doi: 10.1126/science.161.3846.1149.

Abstract

Monospecific antiserum to highly purified second component of human complement (C'2) was used to show the absence of the protein from the serums of four persons homozygous for a hereditary deficiency of second-component activity. Serum from an individual heterozygous for the deficiency contained a reduced amount of this protein as compared to the concentration in normal serum. These observations indicate that genetic deficiency of this component is due to failure of synthesis of normal amounts of the protein rather than to synthesis of an antigenically related, hemolytically inactive analog of C'2.

摘要

用针对高度纯化的人补体第二成分(C'2)的单特异性抗血清,证明了四个遗传性第二成分活性缺乏的纯合子个体的血清中缺乏这种蛋白质。与正常血清相比,来自该缺乏症杂合子个体的血清中这种蛋白质的含量减少。这些观察结果表明,该成分的遗传缺乏是由于正常量蛋白质合成失败所致,而不是合成了与 C'2 抗原相关的、无溶血活性的类似物。

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