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甘露聚糖结合凝集素缺陷症——好消息,坏消息,都无关紧要?

Mannan-binding lectin deficiency - Good news, bad news, doesn't matter?

机构信息

Children's Cancer Research Institute, Vienna, Austria.

出版信息

Clin Immunol. 2012 Apr;143(1):22-38. doi: 10.1016/j.clim.2011.11.002. Epub 2011 Nov 13.

Abstract

Mannan-binding lectin (MBL) deficiency has been classified as a commonly occurring immune disorder, affecting approximately 30% of the human population. MBL, being part of the innate immune system, supports the recognition of infectious pathogens by binding to carbohydrate moieties expressed on microorganisms and activates the lectin pathway of the complement system. MBL2 gene polymorphisms are associated with quantitative and qualitative MBL abnormalities in the serum. The clinical impact of MBL deficiency and its association to a wide variety of diseases has been extensively studied. The picture is puzzling as the studies suggest a detrimental or beneficial or no impact of low or high MBL serum levels on disease susceptibility. In this review we attempt to extract what is relevant from the literature and address controversial issues. We finally suggest that a comprehensive understanding of the role of MBL in human diseases requires considering its context-dependency.

摘要

甘露聚糖结合凝集素(MBL)缺陷被归类为一种常见的免疫紊乱,影响约 30%的人口。MBL 作为先天免疫系统的一部分,通过与微生物表达的碳水化合物部分结合来支持对感染性病原体的识别,并激活补体系统的凝集素途径。MBL2 基因多态性与血清中 MBL 的定量和定性异常有关。MBL 缺乏及其与多种疾病的关联的临床影响已得到广泛研究。研究结果令人困惑,因为这些研究表明,低或高 MBL 血清水平对疾病易感性有不利、有利或无影响。在这篇综述中,我们试图从文献中提取相关内容,并解决有争议的问题。我们最后建议,全面了解 MBL 在人类疾病中的作用需要考虑其上下文相关性。

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