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α1-抗胰蛋白酶(alpha-1-proteinase inhibitor,α1-PI或α1-antitrypsin,α1-AT)是由肝细胞合成的1种丝氨酸蛋白酶抑制剂(serine protease inhibitor,serpin),它最重要的生理功能是在肺内抑制弹性蛋白酶的活性[1]。1963年,Laurell和Eriksson[2]报道了遗传性α1-PI缺乏及其与患者早年(30~40岁)发生肺气肿的关系。流行病学调查显示α1-PI缺乏症
Alpha-1-antitrypsin (α1-PI or α1-antitrypsin, α1-AT) is a serine protease inhibitor synthesized by hepatocytes. The most important physiological Function is to inhibit the activity of elastase in the lung [1]. In 1963, Laurell and Eriksson [2] reported the relationship between hereditary α1-PI deficiency and emphysema in his early years (30-40 years). Epidemiological surveys show α1-PI deficiency