C1-Inhibitor: Structure, Functional Diversity and Therapeutic
Development

doi:10.2174/0929867328666210804085636
摘要

人C1抑制剂（C1INH），也称为C1-酯酶抑制剂，是一种重要的多功能血浆糖蛋白，其独特地参与补体，接触，凝血和纤维蛋白溶解系统的调节网络。C1INH属于丝氨酸蛋白酶抑制剂（serpins）的超家族，对血浆级联的几种靶蛋白酶表现出抑制活性，作为循环中的主要抗炎蛋白起作用。除了其抑制活性外，C1INH还参与与一些内源性蛋白质，多阴离子，细胞和感染因子的非抑制性相互作用。虽然C1INH对多种生理过程至关重要，但它以其遗传性血管性水肿（HAE）的缺陷而闻名，这是一种罕见的常染色体显性遗传性疾病，临床表现为血管通透性增加和水肿的复发性急性发作。自20世纪60年代血浆功能性C1INH缺乏症与HAE之间首次建立联系以来，C1INH的生化表征及其对C1INH依赖性HAE患者替代疗法的治疗开发取得了巨大进展。各种C1INH生物活性，HAE靶向疗法的最新进展以及C1INH商业产品的可用性促使人们深入研究C1INH治疗HAE以外的临床疾病的潜力。本文综述了C1INH的结构和生物学活性，其在HAE发病机制中的作用，以及C1INH研究和治疗开发的最新进展;它还考虑了将C1INH治疗制剂用于血管性水肿以外的应用的一些趋势，从败血症和内毒素休克到COVID-19患者的严重血栓并发症。
关键词: C1抑制剂，C1抑制剂产品，补体，接触系统，遗传性血管性水肿，治疗发展。
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DOI https://dx.doi.org/10.2174/0929867328666210804085636	Print ISSN 0929-8673
Publisher Name Bentham Science Publisher	Online ISSN 1875-533X
当代药物化学

C1抑制剂：结构，功能多样性和治疗发展

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当代药物化学

C1抑制剂：结构，功能多样性和治疗发展

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