Alpha-1-AT is synthesized in the liver and it acts as an inhibitor of proteases such as trypsin, elastase, chymotrypsin, collagenase, leucocytic proteases, plasmin, and thrombin, which may be released during inflammatory reactions in the lung. In the absence of alpha-1-AT, these enzymes are not inhibited and they may digest pulmonary parenchyma. Alpha-1-AT deficiency is associated with chronic obstructive lung disease (emphysema) and less frequently with hepatic cirrhosis in infants and respiratory distress of the newborn. Increase in alpha-1-AT occurs as an acute phase response to tissue necrosis and inflammation. Serum level of alpha-1-AT is elevated in rheumatoid arthritis, bacterial infections, vasculitis, and carcinomatosis.
Protein Aliases: a-1 AT; a1-antitrypsin; alpha 1 antitrypsin; alpha-1 antitrypsin; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Alpha-1-antitrypsin; alpha-1-antitrypsin null; Digestive Zymogen; MGC23330; MGC9222; protease inhibitor 1 (anti-elastase), alpha-1-antitrypsin; Protease Serine 1; serine (or cysteine) proteinase inhibitor, clade A, member 1; Serine Protease 1; Serpin A1; serpin peptidase inhibitor clade A member 1; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1; Short peptide from AAT; SPAAT; TRP1; TRY1
Gene Aliases: A1A; A1AT; AAT; alpha1AT; PI; PI1; PRO0684; PRO2209; PRO2275; SERPINA1
UniProt ID: (Human) P01009
Molecular Function: protease inhibitor