Barszcz D, Zarebska Z, Glińska-Ferenz M, Jabłońska S, Tigałonowa M, Gliński W
Institute of Biochemistry and Biophysics, Polish Academy of Sciences, Warsaw.
Arch Dermatol Res. 1988;280(4):198-206. doi: 10.1007/BF00513958.
The aim of this study was to quantitate the active fraction of the alpha 1-proteinase inhibitor (alpha 1-PI) in psoriasis. Serum proteinase inhibitory capacity was measured vs porcine pancreatic elastase of a known active fraction against its specific substrate (Suc-Ala3-pNA). The inhibitory capacity was determined in 21 symptom-free patients, 134 patients with skin lesions, and 23 healthy volunteers. Alpha 1-PI was found to be significantly decreased in symptom-free patients and in those with stationary lesions, in a manner similar to the reduced activity of neutrophil proteinases, elastase, and cathepsin G. The synthesis of alpha 1-PI was stimulated during the appearance of active psoriatic lesions, but to a much lesser degree in patients with early onset (less than or equal to 21 years) than in patients with late onset of psoriasis (greater than 21 years). The early onset subgroup differed by a more frequent familial occurrence of psoriasis and a more severe course of the disease. The data indicate that the regulation of the proteinase-alpha 1-PI system in psoriasis is abnormal and this may contribute to the pathogenesis of the disease. The decreased alpha 1-PI during flare may be responsible for the disease activity, at least in patients with early onset of psoriasis.
本研究的目的是对银屑病中α1-蛋白酶抑制剂（α1-PI）的活性部分进行定量。针对已知活性部分的猪胰弹性蛋白酶与其特异性底物（Suc-Ala3-pNA），测定血清蛋白酶抑制能力。在21名无症状患者、134名有皮肤损害的患者以及23名健康志愿者中测定了抑制能力。发现无症状患者和静止期损害患者的α1-PI显著降低，其方式类似于中性粒细胞蛋白酶、弹性蛋白酶和组织蛋白酶G活性的降低。在活动性银屑病皮损出现期间，α1-PI的合成受到刺激，但早发型（小于或等于21岁）患者的刺激程度远低于晚发型（大于21岁）银屑病患者。早发型亚组的银屑病家族发病率更高，病程更严重。数据表明，银屑病中蛋白酶-α1-PI系统的调节异常，这可能有助于该疾病的发病机制。至少在早发型银屑病患者中，病情发作期间α1-PI的降低可能是疾病活动的原因。
