Department of Genetics and Clinical Immunology, National Institute of Tuberculosis and Lung Diseases, 01-138 Warsaw, Poland.
Member of the German Center for Lung Research DZL, Department of Respiratory Medicine, Biomedical Research in Endstage and Obstructive Lung Disease Hannover BREATH, 30625 Hannover, Germany.
Int J Mol Sci. 2020 Dec 2;21(23):9187. doi: 10.3390/ijms21239187.
Alpha-1-antitrypsin (AAT), an acute-phase protein encoded by the gene, is a member of the serine protease inhibitor (SERPIN) superfamily. Its primary function is to protect tissues from enzymes released during inflammation, such as neutrophil elastase and proteinase 3. In addition to its antiprotease activity, AAT interacts with numerous other substances and has various functions, mainly arising from the conformational flexibility of normal variants of AAT. Therefore, AAT has diverse biological functions and plays a role in various pathophysiological processes. This review discusses major molecular forms of AAT, including complex, cleaved, glycosylated, oxidized, and S-nitrosylated forms, in terms of their origin and function.
α-1-抗胰蛋白酶(AAT)是一种急性期蛋白,由 基因编码,是丝氨酸蛋白酶抑制剂(SERPIN)超家族的成员。其主要功能是保护组织免受炎症期间释放的酶的侵害,如中性粒细胞弹性蛋白酶和蛋白酶 3。除了其抗蛋白酶活性外,AAT 还与许多其他物质相互作用,具有多种功能,主要源于 AAT 正常变异体的构象灵活性。因此,AAT 具有多种生物学功能,并在各种病理生理过程中发挥作用。本文讨论了 AAT 的主要分子形式,包括复合物、裂解、糖基化、氧化和 S-亚硝酰化形式,及其来源和功能。