Williams D G, Bartlett A, Duffus P
Clin Exp Immunol. 1978 Sep;33(3):425-9.
C3 nephritic factor (C3NeF) activity in sera from three patients with mesangiocapillary glomerulonephritis, one of whom had partial lipodystrophy, was found on chromatography to be associated with fractions containing IgG and no other detectable proteins. Immunoadsorption of IgG from these fractions with a highly purified anti-IgG removed the C3NeF, and the IgG, eluted after combination with the anti-IgG, retained C3NeF activity. In each case the isolated IgG with C3NeF activity was found to contain more than one subclass of IgG and both kappa and lambda chains, indicating that the immunoglobulin comprising C3NeF in these patients is heterogeneous and not monoclonal. The identification of C3NeF as an immunoglobulin suggests that it may be an autoantibody against antigenic determinants of complement components present in the C3 convertase of the alternative pathway.
在3例系膜毛细血管性肾小球肾炎患者(其中1例患有部分脂肪营养不良)的血清中,通过色谱法发现C3肾炎因子(C3NeF)活性与含有IgG且无其他可检测蛋白质的组分相关。用高度纯化的抗IgG对这些组分中的IgG进行免疫吸附可去除C3NeF,与抗IgG结合后洗脱的IgG保留C3NeF活性。在每种情况下,发现具有C3NeF活性的分离出的IgG含有不止一种IgG亚类以及κ链和λ链,这表明这些患者中构成C3NeF的免疫球蛋白是异质性的而非单克隆性的。将C3NeF鉴定为一种免疫球蛋白提示它可能是针对替代途径C3转化酶中存在的补体成分抗原决定簇的自身抗体。