alpha -1 - antitrypsin deficiency
Last reviewed 11/2023
Alpha-1 antitrypsin is a member of the serine protease inhibitor family of plasma proteins.
Alpha-1 antitrypsin has a wide spectrum of antiprotease activity, however, its important physiological role is the inhibition of neutrophil elastase.
Alpha-1 antitrypsin is primarily synthesised in the liver and normally constitutes about 90% of the circulating blood alpha-1 globulin.
Severe alpha-1 antitrypsin deficiency is associated with lung and liver disease.