甘露聚糖结合凝集素相关丝氨酸蛋白酶3的研究现状

Advances in research of mannan-binding lectin-associated serine protease 3

甘露糖结合凝集素相关丝氨酸蛋白酶3(MASP3)是补体凝集素途径的酶成分之一,由MASP1/3基因编码。既往资料证实,MASP3不参与凝集素途径活化,对补体蛋白C3、C4以及C2均无酶切活性,而近期研究表明MASP3是参与补体D因子活化的关键酶,进而可能是联系补体凝集素途径和旁路途径活化的重要成分。此外,人们通过动物疾病模型研究发现MASP3可能作为补体旁路途径异常相关疾病的新治疗靶点,从而为相关疾病提供新的治疗思路。本文旨在对MASP3的编码基因、蛋白结构、生物学功能以及其在旁路途径功能异常相关疾病模型中的研究进展进行综述。

MASP3 is one of the mannan-binding lectin-associated serine proteases（ MASPs） of the complement lectin pathway. It is the alternative splice product of MASP1/3 gene. MASP3 does not participate in the activation of the lectin pathway and it has no cleavage activity towards C2,C4 and C3. Recently,a novel MASP3 function has been discovered that it may play an important role in the alternative pathway through direct activation of factor D. Besides,studies on the murine disease model suggested that MASP3 might be a new therapeutic target for the treatment of alternative pathway-mediated diseases. This review present an overall description of the coding gene and protein structure of MASP3 protein and provide recent research progress on its function and especially in its role in the alternative pathway-mediated diseases.