alpha1-antitrypsin (AAT A1AT) (48D2)

GWB-92B2C9

48D2

Human plasma Composition: HEPES with 0. 15M NaCl 0. 01% BSA 0. 03% sodium azide and 50% glycerolNotes: Western blotting (1:1 000) alpha1-antitrypsin 52kDa is the prototypic member of the serine protease inhibitor (serpin) superfamily which have a major role in inactivating neutrophil elastase and other proteases to maintain proteaseantiprotease balance. The protein is synthesized mainly in the liver but exerts its main function in the lung where it inhibits a variety of serine proteases but primarily neutrophil elastase. It provides more than 90% of the antiproteolytic shield of the lower respiratory tract protecting the elastic tissue from degradation. Disorders of the enzyme include alpha 1-antitrypsin deficiency a hereditary disorder in which lack of alpha 1- antitrypsin leads to uninhibited tissue breakdown during inflammation. This causes pulmonary emphysema and leads to liver cirrhosis in severe cases.

Secreted.

Murine alpha-1-antitrypsin is represented by a cluster of up to 6 individual Serpina1-related genes. The precise complement of Serpina1-related genes present varies according to the strain of the animal.