Product Information
Molecular Weight46737Da
Protein NameAlpha-1-antitrypsin
Human Gene Id5265
Human Swiss Prot NoP01009
SpecificityAAT Polyclonal Antibody detects endogenous levels of AAT protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Concentration1 mg/ml
Storage Stability-20°C/1 year
Other NameSERPINA1; AAT; PI; Alpha-1-antitrypsin; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1
Observed Band(KD)47
Functiondisease:Deficiency of the normal inhibitor in individuals homozygous for allele Z or M-Malton can result in the development of chronic emphysema or infantile liver cirrhosis.,disease:The major physiological function of AAT is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE). A hereditary deficiency of AAT, is associated with a 20-30 fold increased risk of developing chronic obstructive pulmonary disease.,disease:Variant Pittsburgh is the cause of bleeding diathesis.,domain:The reactive center loop (RCL) extends out from the body of the protein and directs binding to the target protease. The protease cleaves the serpin at the reactive site within the RCL, establishing a covalent linkage between the carboxyl group of the serpin reactive site and the serine hydroxyl of the protease. The resulting inactive serpin-protease complex
Subcellular LocationGolgi membrane,extracellular region,proteinaceous extracellular matrix,extracellular space,endoplasmic reticulum,endoplasmic reticulum lumen,Golgi apparatus,ER to Golgi transport vesicle,platelet alpha granule lumen,endoplasmic reticulum-Golg
ExpressionAscites,Bile,Blood,Brain,Cajal-Retzius cell,Colon,Fetal liver,Liver,Lymphoc
Backgroundserpin family A member 1(SERPINA1) Homo sapiens The protein encoded by this gene is secreted and is a serine protease inhibitor whose targets include elastase, plasmin, thrombin, trypsin, chymotrypsin, and plasminogen activator. Defects in this gene can cause emphysema or liver disease. Several transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008],