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C1酯酶抑制剂(C1 esterase inhibitor,C1-INH),又称C1抑制物(C1 inhibitor),是目前已知的唯一一种既能通过经典途径,又能通过凝集素途径对补体系统进行调节的血浆蛋白酶抑制物,其在补体系统、激肽释放酶-激肽系统、纤溶系统和凝血系统中发挥重要的调节作用。目前有4种C1-INH浓缩制剂批准上市,均用于治疗或预防遗传性血管性水肿(hereditary angioedema,HAE)。本文就C1-INH的生化性质与生理功能、相关疾病与临床研究、C1-INH浓缩制剂的制备工艺及产品比较作一综述。
C1 esterase inhibitor (C1-INH), also known as C1 inhibitor, is the only one known to date that can perform both the classical and the lectin pathway on the complement system Regulated plasma protease inhibitors, which play an important regulatory role in the complement system, the kallikrein-kinin system, the fibrinolysis system and the coagulation system. Currently, four C1-INH concentrated formulations are approved for marketing, both for the treatment or prevention of hereditary angioedema (HAE). In this paper, the biochemical properties and physiological functions of C1-INH, related diseases and clinical studies, the preparation process of C1-INH concentrated preparations and their products were reviewed.