Rasche B, Hochstrasser K, Marcic I, Ulmer W T
Respiration. 1975;32(5):340-54. doi: 10.1159/000193664.
In the bronchial mucus of 40 patients with chronic obstructive airway diseases we measured proteolytic activities, the total protein concentrations, alpha1-antitrypsin, alpha1-antichymotrypsin, and the free and bound proteinase inhibitors together with the total proteinase inhibition against trypsin and chymotrypsin. Without exception we always found free proteinase inhibitors together with proteolytic activities. The free-to-bound inhibitor rate was approximately 1:1 alpha1-Antitrypsin and alpha1-antichymotrypsin was measured in sputum only in very low concentrations. One patient with alpha1-anti-trypsin deficiency had no alpha1-antitrypsin, but high concentrations of total proteinase inhibitor-free and bound being in the same relation - in his bronchial mucus. In the alveolar part of the lung, the humoral proteinase inhibitors were effective. In the bronchial part of the lung the specific mucosal inhibitors had the decided importance. The proteinase inhibition of the mucosa-specific inhibitors is probably of great importance for the pathogenesis of airway obstruction, while the humoral proteinase inhibitors are responsible for the pathogenesis of emphysema.
在40例慢性阻塞性气道疾病患者的支气管黏液中,我们测定了蛋白水解活性、总蛋白浓度、α1-抗胰蛋白酶、α1-抗糜蛋白酶,以及游离和结合的蛋白酶抑制剂,同时还测定了对胰蛋白酶和糜蛋白酶的总蛋白酶抑制作用。无一例外,我们总能发现游离蛋白酶抑制剂与蛋白水解活性同时存在。游离抑制剂与结合抑制剂的比例约为1:1。仅在痰液中检测到极低浓度的α1-抗胰蛋白酶和α1-抗糜蛋白酶。1例α1-抗胰蛋白酶缺乏患者的支气管黏液中没有α1-抗胰蛋白酶,但总蛋白酶抑制剂(游离和结合形式)浓度很高,且两者比例相同。在肺的肺泡部分,体液蛋白酶抑制剂发挥作用。在肺的支气管部分,特异性黏膜抑制剂起决定性作用。黏膜特异性抑制剂的蛋白酶抑制作用可能对气道阻塞的发病机制非常重要,而体液蛋白酶抑制剂则与肺气肿的发病机制有关。