Cantin A M, Lafrenaye S, Bégin R O
Unité de Recherche Pulmonaire, Centre Hospitalier Universitaire de Sherbrooke, Quebec, Canada.
Pediatr Pulmonol. 1991;11(3):249-53. doi: 10.1002/ppul.1950110311.
The antigenic concentrations of alpha-1-antitrypsin (alpha 1AT) were measured in 13 patients with cystic fibrosis (CF) and in 11 healthy subjects. Serum alpha 1AT was purified by immunoaffinity chromatography and the antielastase activity of the purified alpha 1AT was determined by measuring the molar ratio necessary to inhibit human neutrophil elastase (HNE). The association rate constant of alpha 1AT with HNE was determined in a timed assay. The capacity of CF serum alpha 1AT to form complexes with porcine pancreatic elastase was studied by polyacrylamide gel electrophoresis. Antigenic concentrations of alpha 1AT mumol/L were markedly increased in the serum of all patients with CF (61.9 +/- 4.3 mumol/L) in comparison to a reference standard (36.7 +/- 1.8 mumol/L; P less than 0.0001). CF serum alpha 1AT was fully active against HNE, and its association rate constant in the presence of HNE was similar to that of healthy subjects. In addition, CF serum alpha 1AT formed complexes with porcine pancreatic elastase that were electrophoretically indistinguishable from those of normal serum alpha 1AT. These results indicate that patients with CF have increased serum alpha 1AT concentrations and that this antiprotease, when purified from serum, functions normally.
对13例囊性纤维化（CF）患者和11名健康受试者的α1 -抗胰蛋白酶（α1AT）抗原浓度进行了测定。血清α1AT通过免疫亲和层析法纯化，纯化后的α1AT的抗弹性蛋白酶活性通过测量抑制人中性粒细胞弹性蛋白酶（HNE）所需的摩尔比来确定。在定时试验中测定α1AT与HNE的缔合速率常数。通过聚丙烯酰胺凝胶电泳研究CF血清α1AT与猪胰弹性蛋白酶形成复合物的能力。与参考标准（36.7±1.8μmol/L；P<0.0001）相比，所有CF患者血清中α1AT的抗原浓度（61.9±4.3μmol/L）显著升高。CF血清α1AT对HNE具有完全活性，其在HNE存在下的缔合速率常数与健康受试者相似。此外，CF血清α1AT与猪胰弹性蛋白酶形成的复合物在电泳上与正常血清α1AT的复合物无法区分。这些结果表明，CF患者血清α1AT浓度升高，并且这种抗蛋白酶从血清中纯化后功能正常。
