人 α-1-抗胰蛋白酶抗体对
01AB03468
Alpha-1-antitrypsin (SERPINA1) is a serine protease inhibitor and a member of the serpin superfamily. This single-chain glycoprotein is primarily synthesized in the liver and secreted into the circulation, though it is also produced in bone marrow and lymphoid tissues. Its primary physiological role is the protection of the lower respiratory tract from proteolytic degradation by neutrophil elastase, as well as other proteases including plasmin, thrombin, and chymotrypsin. Beyond its inhibitory functions, it exhibits significant anti-inflammatory and anti-infective properties. Mutations in the encoding gene lead to alpha-1 antitrypsin deficiency, a condition characterized by protein polymerization in the liver and insufficient pulmonary protection. This deficiency is strongly associated with the development of pulmonary emphysema due to alveolar destruction, liver cirrhosis resulting from polymer accumulation, and systemic inflammatory conditions such as panniculitis.
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技术参数
- 靶点基因名
- SERPINA1
- 基因别名
- AAT; PI
- 蛋白别名
- serpin family A member 1; alpha-1-Antitrypsin
- UniProt
- P01009
- 物种
- Human
产品详情
- 靶点名称
- alpha-1-Antitrypsin
- 蛋白名称
- serpin family A member 1
- 蛋白别名
- Alpha-1-antitrypsin; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1) [Cleaved into: Short peptide from AAT; SPAAT]
- 靶点基因名
- SERPINA1
- 基因别名
- AAT; PI
- UniProt
- P01009
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
- 配方
- TBD
背景介绍
Alpha-1-antitrypsin (SERPINA1) is a serine protease inhibitor and a member of the serpin superfamily. This single-chain glycoprotein is primarily synthesized in the liver and secreted into the circulation, though it is also produced in bone marrow and lymphoid tissues. Its primary physiological role is the protection of the lower respiratory tract from proteolytic degradation by neutrophil elastase, as well as other proteases including plasmin, thrombin, and chymotrypsin. Beyond its inhibitory functions, it exhibits significant anti-inflammatory and anti-infective properties. Mutations in the encoding gene lead to alpha-1 antitrypsin deficiency, a condition characterized by protein polymerization in the liver and insufficient pulmonary protection. This deficiency is strongly associated with the development of pulmonary emphysema due to alveolar destruction, liver cirrhosis resulting from polymer accumulation, and systemic inflammatory conditions such as panniculitis.