Dunlea Danielle M, Fee Laura T, McEnery Thomas, McElvaney Noel G, Reeves Emer P
Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin, Ireland.
J Inflamm Res. 2018 Mar 26;11:123-134. doi: 10.2147/JIR.S156405. eCollection 2018.
Alpha-1 antitrypsin (AAT) is the most abundant serine protease inhibitor circulating in the blood. AAT deficiency (AATD) is an autosomal codominant condition affecting an estimated 3.4 million individuals worldwide. The clinical disease associated with AATD can present in a number of ways including COPD, liver disease, panniculitis and antineutrophil cytoplasmic antibody vasculitis. AATD is the only proven genetic risk factor for the development of COPD, and deficient individuals who smoke are disposed to more aggressive disease. Principally, AAT is a serine protease inhibitor; however, over the past number of years, the assessment of AAT as simply an antiprotease has evolved, and it is now recognized that AAT has significant anti-inflammatory properties affecting a wide range of cells, including the circulating neutrophil.
α1抗胰蛋白酶（AAT）是血液中循环的最丰富的丝氨酸蛋白酶抑制剂。α1抗胰蛋白酶缺乏症（AATD）是一种常染色体共显性疾病，全球约有340万人受其影响。与AATD相关的临床疾病有多种表现形式，包括慢性阻塞性肺疾病（COPD）、肝病、脂膜炎和抗中性粒细胞胞浆抗体血管炎。AATD是COPD发生的唯一经证实的遗传危险因素，吸烟的缺乏个体易患更具侵袭性的疾病。主要地，AAT是一种丝氨酸蛋白酶抑制剂；然而，在过去几年中，对AAT仅作为一种抗蛋白酶的评估已经发展，现在人们认识到AAT具有显著的抗炎特性，可影响包括循环中性粒细胞在内的多种细胞。
