• 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Clinical and Experimental Immunology: An Official Journal of the British Society for Immunology >Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
【24h】

Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy

机译：血清备解素的消耗作为C3肾小球病中C5转化酶异常的生物标志物

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Properdin (P) stabilizes the alternative pathway (AP) convertases, being the only known positive regulator of the complement system. In addition, P is a pattern recognition molecule able to initiate directly the AP on non-self surfaces. Although P deficiencies have long been known to be associated with Neisseria infections and P is often found deposited at sites of AP activation and tissue injury, the potential role of P in the pathogenesis of complement dysregulation-associated disorders has not been studied extensively. Serum P levels were measured in 49 patients with histological and clinical evidence of C3 glomerulopathy (C3G). Patients were divided into two groups according to the presence or absence of C3 nephritic factor (C3NeF), an autoantibody that stabilizes the AP C3 convertase. The presence of this autoantibody results in a significant reduction in circulating C3 (P<0001) and C5 levels (P<005), but does not alter factor B, P and sC5b-9 levels. Interestingly, in our cohort, serum P levels were low in 17 of the 32 C3NeF-negative patients. This group exhibited significant reduction of C3 (P<0001) and C5 (P<0001) and increase of sC5b-9 (P<0001) plasma levels compared to the control group. Also, P consumption was correlated significantly with C3 (r=0798, P=00001), C5 (r=0806, P<00001), sC5b-9 (r=-0683, P=0043) and a higher degree of proteinuria (r=-0862, P=0013). These results illustrate further the heterogeneity among C3G patients and suggest that P serum levels could be a reliable clinical biomarker to identify patients with underlying surface AP C5 convertase dysregulation.
机译：备解素（P）可稳定替代途径（AP）的转化酶，是补体系统中唯一已知的正调节剂。此外，P是一种模式识别分子，能够直接在非自身表面上引发AP。尽管早就知道P缺乏与奈瑟氏菌感染有关，并且经常发现P沉积在AP活化和组织损伤的部位，但是P在与补体失调相关疾病的发病机理中的潜在作用尚未得到广泛研究。在49例具有C3肾小球病（C3G）的组织学和临床证据的患者中测量血清P水平。根据是否存在可稳定AP C3转化酶的自身抗体C3肾病因子（C3NeF），将患者分为两组。该自身抗体的存在导致循环中的C3（P <0001）和C5水平（P <005）显着降低，但不会改变因子B，P和sC5b-9的水平。有趣的是，在我们的队列中，32例C3NeF阴性患者中的17例血清P水平较低。与对照组相比，该组的C3（P <0001）和C5（P <0001）显着降低，而sC5b-9（P <0001）血浆水平升高。而且，磷的消耗与C3（r = 0798，P = 00001），C5（r = 0806，P <00001），sC5b-9（r = -0683，P = 0043）和较高的蛋白尿程度显着相关（ r = -0862，P = 0013）。这些结果进一步说明了C3G患者之间的异质性，并表明P血清水平可能是确定潜在的表面AP C5转化酶失调患者的可靠临床生物标记。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号