Harriman G R, Esser A F, Podack E R, Wunderlich A C, Braude A I, Lint T F, Curd J G
J Immunol. 1981 Dec;127(6):2386-90.
During the routine examination of a healthy 31-yr-old woman, we found an incomplete deficiency of the 9th component of complement (C9). By hemolytic assay her serum C9 activity was 10 to 15% of normal. Limited family studies suggested that she inherited the deficiency as an autosomal codominant trait. She had no history of unusual or severe infections. When tested for bactericidal activity against serum-sensitive Neisseria gonorrhoeae and N. meningitidis, her serum reacted comparably to normal serum. Normal serum depleted immunochemically of C9 and sera from congenitally C9-deficient patients were also bactericidal against serum-sensitive Neisseria but required 120 min to kill the same numbers of gonococci that intact serum killed within 30 min. In the electron microscope, N. gonorrhoeae incubated with C9-depleted serum were fragmented but lacked the typical C lesions. Therefore, serum lacking C9 can kill serum-sensitive Neisseria, unlike sera deficient in the other terminal C components.
在对一名31岁健康女性进行常规检查时，我们发现其补体第9成分（C9）存在不完全缺陷。通过溶血试验，她的血清C9活性为正常水平的10%至15%。有限的家系研究表明，她以常染色体共显性性状遗传了这种缺陷。她没有异常或严重感染的病史。在检测针对血清敏感的淋病奈瑟菌和脑膜炎奈瑟菌的杀菌活性时，她的血清反应与正常血清相当。免疫化学去除C9的正常血清以及先天性C9缺陷患者的血清对血清敏感的奈瑟菌也具有杀菌作用，但杀死相同数量的淋球菌需要120分钟，而完整血清在30分钟内即可完成。在电子显微镜下，与去除C9的血清一起孵育的淋病奈瑟菌发生了碎片化，但没有典型的C损伤。因此，与缺乏其他终末补体成分的血清不同，缺乏C9的血清可以杀死血清敏感的奈瑟菌。
