补体系统
替代补体途径
补语(音乐)
经典补体途径
C3转化酶
补体受体
系数H
补体控制蛋白
CD46型
免疫学
补体成分3
补体因子I
补体因子B
补体膜攻击复合物
炎症
非典型溶血尿毒综合征
生物
细胞生物学
凝集素途径
补体成分5
过敏毒素
丙泊酚
先天免疫系统
补体成分2
免疫系统
遗传学
表型
互补
基因
作者
Jonathan Barratt,Ilene Weitz
标识
DOI:10.3389/fimmu.2021.712572
摘要
The complement system is central to first-line defense against invading pathogens. However, excessive complement activation and/or the loss of complement regulation contributes to the development of autoimmune diseases, systemic inflammation, and thrombosis. One of the three pathways of the complement system, the alternative complement pathway, plays a vital role in amplifying complement activation and pathway signaling. Complement factor D, a serine protease of this pathway that is required for the formation of C3 convertase, is the rate-limiting enzyme. In this review, we discuss the function of factor D within the alternative pathway and its implication in both healthy physiology and disease. Because the alternative pathway has a role in many diseases that are characterized by excessive or poorly mediated complement activation, this pathway is an enticing target for effective therapeutic intervention. Nonetheless, although the underlying disease mechanisms of many of these complement-driven diseases are quite well understood, some of the diseases have limited treatment options or no approved treatments at all. Therefore, in this review we explore factor D as a strategic target for advancing therapeutic control of pathological complement activation.
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