论文部分内容阅读
甘露聚糖结合凝集素（ＭＢＬ）是一种钙离子依赖的Ｃ型血清凝集素，它可以选择性识别结合病原体表面的末端含甘露聚糖、Ｎ－乙酰葡萄糖胺的糖基，通过凝集素通路激活补体，或直接作为调理素介导调理噬菌作用，也可直接与病毒结合，中和病毒并抑制其扩散。ＭＢＬ在天然免疫中起着重要作用，尤其对免疫机能发育尚未成熟的婴幼儿其作用更为重要。ＭＢＬ基因突变是儿童天然免疫缺陷的一个危险因子，有三种突变型：Ｇ５４Ｄ，Ｇ５７Ｅ／Ｑ和 Ｒ５２Ｃ。基因突变破坏了 ＭＢＬ分子的多聚化组装，使其不稳定，易降解，血清中浓度下降，活性受损，患儿可表现出对感染的易感性增加，或伴发其他免疫缺陷病。对严重或频发感染的患儿进行ＭＢＬ血清浓度的检测及其基因突变的筛查，有助于诊断和治疗。国外已报道用纯化正常人血浆ＭＢＬ治疗ＭＢＬ遗传缺陷，重建调理噬菌功能。
Mannan-binding lectin (MBL) is a calcium-dependent serum type C lectin that selectively recognizes glycosyl groups that bind to the terminal mannan-containing, N-acetylglucosamine at the surface of the pathogen and passes through the lectin pathway Activation of complement, or mediated directly as a regulator of phagocytosis, can also be directly associated with the virus, neutralize the virus and inhibit its proliferation. MBL plays an important role in innate immunity, especially for infants and young children whose immune function is immature. MBL mutations are a natural risk factor for childhood natural immune defects, there are three mutations: G54D, G57E / Q and R52C. Mutation of MBL molecules undermines the multimeric assembly of MBL molecules, making them unstable, easily degraded, their serum concentrations declining, and their activity impaired. Children may show increased susceptibility to infection or other immunodeficiency disorders. Serum MBL serum concentrations in children with severe or frequent infection screening and genetic mutation screening help diagnosis and treatment. Abroad has been reported with purified normal human plasma MBL treatment of genetic defects in MBL reconstruction reconstruction phagocytosis.