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C_1~--INH是属于丝氨酸蛋白酶抑制剂(即属于Serpin基因家族),含糖量高,为单一多肽链。活性中心位于C~-端。有8种变异型。先天性缺乏C_1~1-INH主要见于遗传性血管神经性水肿。某些严重疾病也会造成后天性C_1~--INH缺乏症。另一些病也会使血液中C_1~--INH含量升高。现发现体内至少有4种细胞合成和分泌C_1~--INH,C_1~--INH基因位于第11条染色体上,具有限制性片断长度多态性。除用同化激素外,C_1~--INH浓制剂、干扰素以及肿瘤坏死因子在治疗和预防C_1~--INH缺乏症方面有很好的效果。
C_1~--INH is a serine protease inhibitor (which belongs to the Serpin gene family) and has a high sugar content and is a single polypeptide chain. The active center is located at the C-terminus. There are 8 variants. Congenital absence of C_1~1-INH is mainly seen in hereditary angioneurotic edema. Some serious diseases can also cause acquired C_1~-INH deficiency. Other diseases also increase the C1~--INH levels in the blood. It has been found that at least 4 cells in the body synthesize and secrete C_1~--INH, and the C_1~-INH gene is located on the 11th chromosome with restriction fragment length polymorphism. In addition to anabolic hormones, C_1~--INH concentrated preparations, interferon and tumor necrosis factor have a good effect in the treatment and prevention of C_1~--INH deficiency.