Barton P A, Warren J S
Department of Pathology, University of Michigan Medical School, Ann Arbor 48109-0602.
Infect Immun. 1993 Apr;61(4):1474-81. doi: 10.1128/iai.61.4.1474-1481.1993.
Patients with disseminated Neisseria meningitidis infections (meningococcemia) suffer from a fulminant shock syndrome that is accompanied by extraordinarily high concentrations in serum of tumor necrosis factor (TNF). People with homozygous deficiencies of late complement components (C5, C6, C7, and C8) experience a high incidence of disseminated neisserial infections yet suffer from an attenuated form of the disease. The mechanisms that account for this disparity in host response are unclear, but they may in part be related to differences in the systemic TNF response that are modulated by terminal complement components (C5 to C9). The role of C5 in the modulation of the systemic endotoxin-induced TNF response was studied with matched strains of C5-deficient (B10 D2/Osn) and complement-sufficient (B10 D2/Nsn) mice. Following lipopolysaccharide (LPS) administration, complement-sufficient mice exhibited more rapid increases in pulmonary and hepatic vascular permeabilities than did C5-deficient controls. Complement-sufficient mice developed acute passive hepatic congestion, they appeared more ill than C5-deficient mice, and they exhibited a twofold greater rise in serum TNF activity compared with that by C5-deficient mice. C5-deficient mice reconstituted with normal serum before an LPS injection exhibited pulmonary and hepatic vascular permeability increases and serum TNF levels approaching those observed in complement-sufficient mice. Alveolar and peritoneal macrophages isolated from complement-sufficient and C5-deficient mice and incubated in heat-inactivated serum did not exhibit differences in TNF mRNA expression or secreted TNF activity following stimulation with LPS. However, incubation of macrophages in complement-sufficient mouse serum (before LPS stimulation) resulted in increased TNF mRNA expression and TNF activity compared with those in cells incubated in C5-deficient serum. In vitro studies employing human complement components and peripheral blood monocytes revealed that recombinant C5a, in the presence or absence of LPS, can induce increased concentrations of TNF and that C5b to C9 had no additional modulatory effect on the TNF response. These data suggest that C5 modulates the endotoxin-triggered TNF response. The role of complement components distal to C5 (i.e., C5b to C9) in the endotoxin-triggered TNF response remains unclear.
播散性脑膜炎奈瑟菌感染(脑膜炎球菌血症)患者会患上暴发性休克综合征,其血清中肿瘤坏死因子(TNF)浓度异常高。晚期补体成分(C5、C6、C7和C8)纯合缺陷的人发生播散性奈瑟菌感染的发生率很高,但病情较轻。宿主反应存在这种差异的机制尚不清楚,但可能部分与由末端补体成分(C5至C9)调节的全身TNF反应差异有关。利用C5缺陷(B10 D2/Osn)和补体充足(B10 D2/Nsn)的匹配小鼠品系,研究了C5在调节全身内毒素诱导的TNF反应中的作用。给予脂多糖(LPS)后,补体充足的小鼠肺和肝血管通透性的增加比C5缺陷对照组更快。补体充足的小鼠出现急性被动性肝充血,它们看起来比C5缺陷小鼠病情更重,并且与C5缺陷小鼠相比,其血清TNF活性升高了两倍。在注射LPS前用正常血清重建的C5缺陷小鼠,其肺和肝血管通透性增加,血清TNF水平接近补体充足小鼠中观察到的水平。从补体充足和C5缺陷小鼠中分离出的肺泡巨噬细胞和腹腔巨噬细胞,在热灭活血清中培养,在用LPS刺激后,TNF mRNA表达或分泌的TNF活性没有差异。然而,与在C5缺陷血清中培养的细胞相比,在补体充足小鼠血清中培养巨噬细胞(在LPS刺激前)导致TNF mRNA表达和TNF活性增加。利用人补体成分和外周血单核细胞进行的体外研究表明,重组C5a在有或没有LPS的情况下都能诱导TNF浓度增加,并且C5b至C9对TNF反应没有额外的调节作用。这些数据表明C5调节内毒素触发的TNF反应。C5远端的补体成分(即C5b至C9)在内毒素触发的TNF反应中的作用仍不清楚。