Nilsson U R, Müller-Eberhard H J
J Exp Med. 1967 Jan 1;125(1):1-16. doi: 10.1084/jem.125.1.1.
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.
已证明某些近交系小鼠的遗传性补体缺陷是由于补体第五成分的单独缺乏所致。蛋白质MuB1（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，经免疫化学分析表明它与人补体第五成分（C'5）相关。小鼠抗MuB1可特异性抑制人血清中的C'5溶血活性，而小鼠抗人C'5抗血清可抑制正常小鼠血清中的C'5溶血活性。高度纯化的人C'5可恢复补体缺陷小鼠血清的溶血活性。因此，可以得出结论，hc'或MuB1构成了人补体第五成分的小鼠类似物。发现正常小鼠血清中MuB1的浓度存在性别相关差异。通过定量沉淀分析表明，雄性小鼠血清中的MuB1含量是雌性小鼠的两倍。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也存在性别相关差异。
