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补体激活以及在原发性混合性冷球蛋白血症中溶解免疫复合物或阻止其形成的能力受损。

Complement activation and impaired capacity to solubilize immune complexes or to prevent their formation in essential mixed cryoglobulinemia.

作者信息

Corvetta A, Spaeth P J, Ghirelli P A, Orecchioni F, Buetler R, Montroni M, Nydegger U E

出版信息

Diagn Immunol. 1983;1(4):315-23.

PMID:6333960
Abstract

The complement profile, the immune complex solubilizing capacity (ICSC), the immune complex precipitation inhibition capacity (ICPIC), the presence of cryoprecipitable material, and the presence of immune-aggregate- and non-immune-aggregate mediated C1q-binding activity was assessed in serum samples from 23 patients suffering from essential mixed cryoglobulinemia (EMC). No correlation between the levels of cryoglobulins and the clinical activity of EMC was found. The mean C1q-binding activity in EMC serum samples was abnormally elevated 28 +/- 29% (mean +/- SD). In six out of eight serum samples that contained C1q-binding material, evidence was obtained that such material was of the complexed immunoglobulin G (IgG) type. Among the levels of C1q, C1r, C2, C4, C3, C5, C6, C1-inhibitor, C3d, B, I, H, as well as the total hemolytic activity and the activity of the alternative pathway of the complement, the mean serum concentrations of C1, C2, and C4 and in consequence the mean total hemolytic activity were significantly reduced, whereas the mean levels of C3d were significantly elevated in the EMC serum samples. The capacity of the 23 EMC serum samples to solubilize preformed immune precipitates from bovine serum albumin (BSA) and rabbit anti-BSA antibodies as well as their capacity to prevent the formation of the precipitable form of such complexes was analyzed. Compared to the ICSC and the ICPIC of 30 normal human sera, the ICSC and the ICPIC of EMC serum samples were reduced to 57 +/- 24% and 38 +/- 33%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

对23例原发性混合性冷球蛋白血症(EMC)患者的血清样本进行了补体谱、免疫复合物溶解能力(ICSC)、免疫复合物沉淀抑制能力(ICPIC)、冷沉淀物质的存在情况以及免疫聚集体和非免疫聚集体介导的C1q结合活性的评估。未发现冷球蛋白水平与EMC临床活性之间存在相关性。EMC血清样本中的平均C1q结合活性异常升高28±29%(平均值±标准差)。在8份含有C1q结合物质的血清样本中,有6份获得证据表明此类物质为复合免疫球蛋白G(IgG)型。在C1q、C1r、C2、C4、C3、C5、C6、C1抑制剂、C3d、B、I、H的水平以及补体的总溶血活性和替代途径活性中,EMC血清样本中C1、C2和C4的平均血清浓度以及相应的平均总溶血活性显著降低,而C3d的平均水平显著升高。分析了23份EMC血清样本溶解牛血清白蛋白(BSA)和兔抗BSA抗体预先形成的免疫沉淀物的能力以及它们阻止此类复合物形成可沉淀形式的能力。与30份正常人血清的ICSC和ICPIC相比,EMC血清样本的ICSC和ICPIC分别降至57±24%和38±33%。(摘要截取自250字)

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引用本文的文献

1
Extreme hypercomplementemia in the setting of mixed cryoglobulinemia.混合性冷球蛋白血症时的极度高补体血症。
Clin Rheumatol. 2011 Mar;30(3):415-8. doi: 10.1007/s10067-010-1652-7. Epub 2011 Jan 7.
2
The kidney in mixed cryoglobulinemias.混合性冷球蛋白血症中的肾脏。
Springer Semin Immunopathol. 1987;9(4):395-415. doi: 10.1007/BF00197217.
3
Formation of soluble immune complexes by complement in sera of patients with various hypocomplementemic states. Difference between inhibition of immune precipitation and solubilization.
补体在各种低补体血症状态患者血清中形成可溶性免疫复合物。免疫沉淀抑制与溶解之间的差异。
J Clin Invest. 1985 Dec;76(6):2127-33. doi: 10.1172/JCI112217.
4
Cold-dependent activation of complement: recognition, assessment, and mechanism.补体的冷依赖性激活:识别、评估及机制
J Clin Immunol. 1992 Sep;12(5):362-70. doi: 10.1007/BF00920794.