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CFB P00751

补体因子B (EC 3.4.21.47) (C3/C5转化酶) (富含甘氨酸的β糖蛋白) (GBG) (PBF2) (备解素因子B) [裂解为:补体因子B Ba (Ba);补体因子B Bb (Bb)]

蛋白质信息 (UniProt)

补体因子B (EC 3.4.21.47) (C3/C5转化酶) (富含甘氨酸的β糖蛋白) (GBG) (PBF2) (备解素因子B) [裂解为:补体因子B Ba (Ba);补体因子B Bb (Bb)]

P00751

功能描述

补体系统替代途径C3和C5转化酶复合物催化组分的前体,该系统是一系列导致病原体吞噬和分解以及增强适应性免疫系统信号的蛋白级联反应 (PubMed:3638964, PubMed:624565, PubMed:6554279, PubMed:6919543, PubMed:9748277)。替代补体途径作为一个放大环,通过促进额外C3和C5转化酶的形成来增强其他补体途径(经典途径、凝集素途径和GZMK途径) (PubMed:3638964, PubMed:624565, PubMed:6554279, PubMed:6919543, PubMed:9748277)。CFB被CFD切割并激活,生成Ba和Bb链;Bb链构成C3和C5转化酶的催化组分 (PubMed:6769474, PubMed:9748277)。 {ECO:0000269|PubMed:3638964, ECO:0000269|PubMed:624565, ECO:0000269|PubMed:6554279, ECO:0000269|PubMed:6769474, ECO:0000269|PubMed:6919543, ECO:0000269|PubMed:9748277}.; FUNCTION: [Complement factor B Bb]: 替代补体途径补体C3和C5转化酶复合物的丝氨酸蛋白酶组分 (PubMed:30643019, PubMed:3638964, PubMed:624565, PubMed:6554279, PubMed:6919543)。经因子D (CFD) 切割激活后,与补体C3b共同形成C3转化酶 (PubMed:3638964, PubMed:624565, PubMed:6554279, PubMed:6919543, PubMed:9748277)。作为C3转化酶的一部分,将C3切割并激活为C3a过敏毒素和C3b调理素,即补体途径的下一个组分 (PubMed:3638964, PubMed:624565, PubMed:6554279, PubMed:6919543, PubMed:9748277)。当额外的补体C3b分子与C3转化酶结合时,形成C5转化酶,将C5切割并激活为C5a过敏毒素和膜攻击复合物的C5b组分 (PubMed:30643019, PubMed:624565, PubMed:6554279)。 {ECO:0000269|PubMed:30643019, ECO:0000269|PubMed:3638964, ECO:0000269|PubMed:624565, ECO:0000269|PubMed:6554279, ECO:0000269|PubMed:6919543, ECO:0000269|PubMed:9748277}.; FUNCTION: [Complement factor B Ba]: 参与预激活B淋巴细胞的增殖和分化、外周血单核细胞的快速铺展、刺激淋巴细胞母细胞化以及红细胞溶解。 {ECO:0000269|PubMed:3486249}.

亚细胞定位

Secreted

关键词

3D-structure Age-related macular degeneration Alternative splicing Cleavage on pair of basic residues Complement alternate pathway Direct protein sequencing Disease variant Disulfide bond Glycation Glycoprotein