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Alpha-1 antitrypsin in autoimmune diseases: Roles and therapeutic prospects
Rui Sun1, Zhiqiang Xu2, Chenxi Zhu1
1Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China; Clinical Institute of Inflammation and Immunology (CIII), Frontiers Science Center for Disease-related Molecular Network, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Abstract:
Alpha-1 antitrypsin (A1AT) is a protease inhibitor in the serum. Its primary function is to inhibit the activity of a series of proteases, including proteinase 3, neutrophil elastase, metalloproteases, and cysteine-aspartate proteases. In addition, A1AT also has anti-inflammatory, anti-apoptotic, anti-oxidative stress, anti-viral, and anti-bacterial activities and plays essential roles in the regulation of tissue repair and lymphocyte differentiation and activation. The overactivation of the immune system characterizes the pathogenesis of autoimmune diseases. A1AT treatment shows beneficial effects on patients and animal models with autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus. This review summarizes the functions and therapeutic prospects of A1AT in autoimmune diseases.
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