|Reactivity||Human, Mouse, Rat|
|Calculated MW||46737 Da|
|Other Names||Alpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1, AAT, PI|
|Formulation||100 µg (0.5 mg/ml) of antibody in PBS pH 7.2, 0.01 % BSA, 0.03 % ProClin®, and 50 % glycerol.|
|Handling||The antibody solution should be gently mixed before use.|
|Precautions||Alpha1 Antitrypsin Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. Irreversibly inhibits trypsin, chymotrypsin and plasminogen activator. The aberrant form inhibits insulin-induced NO synthesis in platelets, decreases coagulation time and has proteolytic activity against insulin and plasmin.|
|Cellular Location||Secreted. Endoplasmic reticulum. Note=The S and Z allele are not secreted effectively and accumulate intracellularly in the endoplasmic reticulum|
|Tissue Location||Ubiquitous. Expressed in leukocytes and plasma.|
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Alpha 1 Antitrypsin is an acute-phase plasma protein found at 290 mg per 100 ml. It functions as a serine protease inhibitor with targets like elastase, plasmin, collagenase, thrombin, leucocyte proteases, trypsin, chymotrypsin, and plasminogen activator. Clinically, its deficiency is associated with two major diseases: pulmonary emphysema and early onset/juvenile hepatic cirrhosis. It is elevated in inflammatory conditions, malignancies, liver disease and pregnancy and also after surgical trauma and use of oral contraceptives. The simultaneous quantitative determination of alpha-1-PI and ceruloplasmin permits differential diagnosis of liver afflictions. Rise in alpha 1 antitrypsin occurs as an acute phase response to tissue necrosis and inflammation.
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