Deficiency of the fifth component of complement in mice with an inherited complement defect.
Deficiency of the fifth component of complement in mice with an inherited complement defect.
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DOI：
10.1084/jem.125.1.1
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发表时间：
1967-01-01
期刊：
影响因子：
--
通讯作者：
Müller-Eberhard HJ
中科院分区：
文献类型：
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作者：
Nilsson UR;Müller-Eberhard HJ
某些近交系小鼠的遗传性补体缺乏症被证明是由于补体的第五种成分的单独缺乏。MuB 1蛋白（或hc '）存在于正常小鼠血清中，但在补体缺陷小鼠的血清中不存在，研究表明该蛋白与人类补体的第五组分（C' 5）具有免疫化学相关性。C'5溶血活性在人血清中被小鼠抗MuB 1特异性抑制，在正常小鼠血清中被抗人C'5的小鼠抗血清特异性抑制。高度纯化的人C '5重建补体缺陷小鼠血清的溶血活性。因此，得出结论，he ′或MuB 1构成人补体第五组分的鼠类似物。正常小鼠血清中MuB1浓度存在性别差异。通过沉淀素定量分析，证实雄性小鼠血清中MuB 1的含量是雌性小鼠血清中MuB 1的两倍。通过溶血试验也检测到了C ′ 5浓度的这种差异。此外，还发现C '6和C' 7也受到与性别相关的变异。
The inherited complement deficiency of certain inbred strains of mice was shown to be due to an isolated lack of the fifth component of complement. The protein MuB1 (or hc'), which is present in normal mouse serum but absent from the serum of complement-deficient mice, was shown to be immunochemically related to the fifth component of human complement (C'5). C'5 hemolytic activity was specifically inhibited in human serum by mouse anti-MuB1 and in normal mouse serum by mouse antiserum to human C'5. Highly purified human C'5 reconstituted the hemolytic activity of complement-deficient mouse serum. It was, therefore, concluded that he', or MuB1, constitutes the murine analogue of the fifth component of human complement. The MuB1 concentration in normal mouse serum was found to be subject to a sex-related variation. By quantitative precipitin analysis it was demonstrated that serum from male mice contains twice as much MuB1 as that of female mice. This difference in C'5 concentration was also detected by hemolytic assay. In addition, C'6 and C'7 were also found to be subject to sex-related variations.