Spitzer R E, Stitzel A E, Tsokos G
Department of Pediatrics, SUNY Health Science Center, Syracuse 13210.
Clin Immunol Immunopathol. 1992 Sep;64(3):177-83. doi: 10.1016/0090-1229(92)90197-v.
The antibody to the alternative pathway C3 convertase, designated C3 nephritic factor or C3NeF, is an autoantibody that is produced in everyone from the time of birth. The elaboration of C3NeF utilizes germline V-region genes which undergo antigen-driven affinity maturation, resulting in an autoantibody that is produced in large amounts with high affinity and narrow specificity. Our data also suggest that under normal conditions, the idiotypic network may play an important part in the control of this autoantibody. Further, a defect in the network with loss of control or inappropriate stimulation may be an underlying mechanism in the unrestricted production of C3NeF in patients with membranoproliferative glomerulonephritis.
针对替代途径C3转化酶的抗体，称为C3肾炎因子或C3NeF，是一种自出生起就存在于每个人体内的自身抗体。C3NeF的产生利用种系V区基因，这些基因经历抗原驱动的亲和力成熟，产生大量具有高亲和力和狭窄特异性的自身抗体。我们的数据还表明，在正常情况下，独特型网络可能在这种自身抗体的控制中起重要作用。此外，网络缺陷导致控制丧失或刺激不当可能是膜增生性肾小球肾炎患者中C3NeF不受限制产生的潜在机制。
