Goldsmith G H, Stern R C, Saito H, Ratnoff O D
J Lab Clin Med. 1977 Jan;89(1):131-4.
Previous investigators have suggested that the biological activity of plasma prekallikrein is defective in cystic fibrosis. In contrast, no such difference was demonstrable between normal and cystic fibrosis plasma. Esterolytic activity for the synthetic substrate p-toluene sulfonyl-arginine methyl ester (TAMe) evolved normally in cystic fibrosis plasma treated with chloroform and ellagic acid, a measure of generation of plasma kallikrein. Additionally, plasma prekallikrein (Fletcher factor) and high molecular weight kininogen (Fitzgerald factor), a substrate of plasma kallikrein, were normal. Thus, the concept that cystic fibrosis is associated with abnormalities in the plasma kallikrein-kinin system could not be supported.
先前的研究人员曾提出，囊性纤维化患者血浆前激肽释放酶的生物活性存在缺陷。然而，正常血浆与囊性纤维化血浆之间并未显示出这种差异。用氯仿和鞣花酸处理囊性纤维化血浆后，其对合成底物对甲苯磺酰精氨酸甲酯（TAMe）的酯解活性正常发展，这是血浆激肽释放酶生成的一种衡量指标。此外，血浆前激肽释放酶（弗莱彻因子）和高分子量激肽原（菲茨杰拉德因子，血浆激肽释放酶的一种底物）均正常。因此，囊性纤维化与血浆激肽释放酶 - 激肽系统异常相关这一概念无法得到支持。
