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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Anti-C1q autoantibodies from systemic lupus erythematosus patients activate the complement system via both the classical and lectin pathways
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Anti-C1q autoantibodies from systemic lupus erythematosus patients activate the complement system via both the classical and lectin pathways

机译：系统性红斑狼疮患者的抗C1q自身抗体通过经典途径和凝集素途径激活补体系统

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摘要

Autoantibodies against complement C1q (anti-C1q) strongly correlate with the occurrence of lupus nephritis and hypocomplementemia in systemic lupus erythematosus (SLE). Although a direct pathogenic role of anti-C1q has been suggested, the assumed complement-activating capacity remains to be elucidated. Using an ELISA-based assay, we found that anti-C1q activate the classical (CP) and lectin pathways (LP) depending on the anti-C1q immunoglobulin-class repertoire present in the patient's serum. IgG anti-C1q resulted in the activation of the CP as reflected by C4b deposition in the presence of purified Cl and C4 in a dose-dependent manner. The extent of C4b deposition correlated with anti-C1q levels in SLE patients but not in healthy controls. Our data indicate that SLE patient-derived anti-C1q can activate the CP and the LP but not the alternative pathway of complement. These findings are of importance for the understanding of the role of anti-C1q in SLE suggesting a direct link to hypocomplementemia. (C) 2015 Elsevier Inc. All rights reserved.
机译：抗补体C1q（抗C1q）的自身抗体与系统性红斑狼疮（SLE）中狼疮性肾炎和低补体血症的发生密切相关。尽管已提出抗C1q的直接致病作用，但尚需阐明假定的补体激活能力。使用基于ELISA的检测方法，我们发现抗C1q激活经典（CP）和凝集素途径（LP），具体取决于患者血清中存在的抗C1q免疫球蛋白类库。 IgG抗C1q导致CP的活化，如在纯化的C1和C4的存在下以剂量依赖性方式由C4b沉积所反映。在SLE患者中，C4b沉积的程度与抗C1q水平相关，但在健康对照组中则不相关。我们的数据表明，来自SLE患者的抗C1q可以激活CP和LP，但不能激活补体的替代途径。这些发现对于理解抗C1q在SLE中的作用非常重要，表明与低补血症的直接联系。 （C）2015 Elsevier Inc.保留所有权利。

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