Complement constitutes a major part of the innate immune system. The study of complement in human health has historically focused on infection risks associated with complement protein deficiencies; however, recent interest in the field has focused on overactivation of complement as a cause of immune injury and the development of anticomplement therapies to treat human diseases. The kidneys are particularly sensitive to complement injury, and anticomplement therapies for several kidney diseases have been investigated. Overactivation of complement can result from loss-of-function mutations in complement regulators; gain-of-function mutations in key complement proteins such as C3 and factor B; or autoantibody production, infection, or tissue stresses, such as ischemia and reperfusion, that perturb the balance of complement activation and regulation. Here, we provide a high-level review of the status of anticomplement therapies, with an emphasis on the transition from rare diseases to more common kidney diseases.

中文翻译：

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抗补体治疗适应症从罕见遗传性疾病扩展到常见肾脏疾病

补体构成先天免疫系统的主要部分。人类健康中的补体研究历来侧重于与补体蛋白缺乏相关的感染风险。然而，最近该领域的兴趣集中在补体过度激活作为免疫损伤的原因以及开发治疗人类疾病的抗补体疗法。肾脏对补体损伤特别敏感，并且已经研究了针对多种肾脏疾病的抗补体疗法。补体过度激活可能是由于补体调节因子功能缺失突变所致；关键补体蛋白（如 C3 和 B 因子）的功能获得性突变；自身抗体的产生、感染或组织应激（例如缺血和再灌注）会扰乱补体激活和调节的平衡。在这里，我们对抗补体疗法的现状进行了高水平的回顾，重点是从罕见疾病到更常见肾脏疾病的转变。