Nemerow G R, Gewurz H, Osofsky S G, Lint T F
J Clin Invest. 1978 Jun;61(6):1602-10. doi: 10.1172/JCI109080.
A 46-yr-old female with chronic pyelonephritis was found to lack complement (C) activity by the use of hemolytic screen assays in agarose gels. These assays also revealed a propensity of patient serum to form an activated complex of the fifth and sixth components of C, C56. Each of the C component hemolytic activities was present in normal or elevated amounts with the exception of C7, which was undetectable; addition of purified C7 led to the restoration of hemolytic activity. C-dependent phagocytosis, immune adherence, and neutrophil chemotaxis were normal. Family studies demonstrated that the defect was transmitted as an autosomal codominant apparently not linked with alleles at the HLA-A or HLA-B loci. Persisting C56 was readily formed in this as compared to normal serum upon incubation with multiple C activators including zymosan, inulin, immune complexes, heat-aggregated human gamma globulin, endotoxin, and agarose. A heat-stable (56 degrees C, 30 min) activity which consumed C7 with time-and temperature-dependent kinetics was detected in plasma and serum, and seemed to be similar to a "C7 inactivator" previously described in another C7-deficient individual. However, this activity was found to have properties identical to those of C56 during low ionic strength precipitation and chromatography on Sephadex G-200, to be specifically removed upon passage through an anti-C5 immunoadsorbent column, and to be associated with a small amount of C56, suggesting that it represents an expression of small amounts of C56 rather than a new C-inhibitory activity. Thus, an individual with chronic nephritis lacking C7 is reported; the utility of a hemolytic screen assay in agarose plates for the detection of such patients is emphasized; persisting C56 is shown readily to be formed in this serum; and the presence of C7-consuming activity which is associated with and in all likelihood attributable to C56 is shown.
一名46岁患有慢性肾盂肾炎的女性，通过琼脂糖凝胶溶血筛查试验发现其缺乏补体（C）活性。这些试验还显示患者血清有形成补体第五和第六成分（C56）活化复合物的倾向。除了无法检测到的C7外，每种补体成分的溶血活性均正常或升高；添加纯化的C7可恢复溶血活性。补体依赖的吞噬作用、免疫黏附及中性粒细胞趋化性均正常。家系研究表明，该缺陷以常染色体共显性方式遗传，显然与HLA - A或HLA - B位点的等位基因无关。与正常血清相比，该血清在与包括酵母聚糖、菊粉、免疫复合物、热聚集人γ球蛋白、内毒素和琼脂糖在内的多种补体激活剂孵育后，很容易形成持续存在的C56。在血浆和血清中检测到一种热稳定（56℃，30分钟）的活性，其随时间和温度依赖性动力学消耗C7，似乎与先前在另一名C7缺陷个体中描述的“C7灭活剂”相似。然而，在低离子强度沉淀和Sephadex G - 200柱层析过程中，发现该活性与C56具有相同的性质，通过抗C5免疫吸附柱后可被特异性去除，并与少量C56相关联，这表明它代表少量C56的一种表现形式，而非一种新的补体抑制活性。因此，报道了一名缺乏C7的慢性肾炎患者；强调了琼脂糖平板溶血筛查试验在检测此类患者中的实用性；显示该血清中很容易形成持续存在的C56；并显示存在与C56相关且很可能归因于C56的C7消耗活性。
