Böttger E C, Metzger S, Bitter-Suermann D, Stevenson G, Kleindienst S, Burger R
Eur J Immunol. 1986 Oct;16(10):1231-5. doi: 10.1002/eji.1830161008.
A recently described genetically controlled C3 deficiency (C3D) in guinea pigs (GP) provided a unique model for studying the role of C3 in the afferent limb of the humoral immune response in a direct manner. These C3D animals, which have only 5-7% of normal serum C3 level, were immunized with the bacteriophage phi chi 174, a T cell-dependent antigen, followed by a booster injection after 4 weeks (1.5 X 10(9) plaque-forming units/kg). The formation of IgM and IgG antibody in the course of the primary and secondary response was determined and compared with a control group of inbred strain 2 GP. The C3D animals showed a markedly diminished antibody response to this antigen. Amplification of the antibody titer as well as regular isotype switching from IgM to IgG was absent in the secondary response. Increasing the amount of antigen to a high dose (1 X 10(10) plaque-forming units/kg) led to a normalization of the antibody response. The impairment in antibody formation resembles closely the impaired antibody response in C4-deficient or C2-deficient GP, which both have a block in activation of C3 via the classical pathway. However, in contrast to C4D GP or C2D GP the C3D GP do not exhibit serological characteristics of immune complex disease. They have normal levels of total serum IgM, of IgM anti-2,4-dinitrophenyl antibodies and of IgM rheumatoid factors.
最近在豚鼠中发现的一种基因控制的C3缺陷(C3D)为直接研究C3在体液免疫反应传入支中的作用提供了一个独特的模型。这些C3D动物的血清C3水平仅为正常水平的5%-7%,用噬菌体phi chi 174(一种T细胞依赖性抗原)进行免疫,4周后进行加强注射(1.5×10⁹噬斑形成单位/千克)。测定了初次和二次反应过程中IgM和IgG抗体的形成,并与近交系2豚鼠的对照组进行了比较。C3D动物对该抗原的抗体反应明显减弱。二次反应中抗体滴度没有放大,也没有从IgM到IgG的正常同种型转换。将抗原量增加到高剂量(1×10¹⁰噬斑形成单位/千克)可使抗体反应恢复正常。抗体形成的受损与C4缺陷或C2缺陷豚鼠中受损的抗体反应非常相似,这两种豚鼠都通过经典途径阻断了C3的激活。然而,与C4D豚鼠或C2D豚鼠不同,C3D豚鼠没有表现出免疫复合物疾病的血清学特征。它们的血清总IgM、IgM抗2,4-二硝基苯基抗体和IgM类风湿因子水平正常。