基因名:
MASP2
产品别名:
MAP-2; MAP19; MASP-2; MASP1P1; sMAP; MASP2; MBL associated serine protease 2; MBL associated serine protease 2; mannan-binding lectin serine protease 2; MBL-associated plasma protein of 19 kD; mannan binding lectin serine peptidase 2; mannan-binding lectin serine peptidase 1 pseudogene 1; mannan-binding lectin serine protease 1 pseudogene 1; mannose-binding lectin associated protein 19; mannose-binding lectin-associated serine protease 2; mannose-binding protein-associated serine protease 2; small MBL-associated protein; 甘露聚糖结合凝集素丝氨酸肽酶2; 甘露聚糖结合凝集素丝氨酸肽酶2蛋白; 甘露聚糖结合凝集素关联丝氨酸蛋白酶2(MASP2);
背景信息:
MASP2, MBL associated serine protease 2, is a serum protease that plays an important role in the activation of the complement system via mannose-binding lectin (MBL). Binding of MBL to carbohydrate groups triggers MASP2 activation by auto-catalytic cleavage which leads to cleavage of C2 and C4, leading to their activation and to the formation of C3 convertase. MASP2 also binds to L-Ficolin and H-Ficolin, and when the complex binds pathogens the MASP is activated. Low MBL levels in serum are associated with increased susceptibility to infection, and an Asp105Gly mutation in MASP2 is associated with low MASP2 levels in serum. Reduced functional levels of MBL are also postulated to be associated with generation of autoantibodies in systemic lupus erythematosus, acting through a decreased clearance of apoptotic material. A truncated form of MASP2 that constitutes the amino 185 residues, Map19 is also a component of the MBL-MASP-1 complex.