Alpha-1-Antitrypsin Active Protein | A1AT active protein
Alpha-1-Antitrypsin (A1AT), Human Serum
Custom preparations, technical support, bulk quantities and aliquoting available.
Human alpha 1 Antitrypsin or Human A1-antitrypsin (A1AT), also known as serum trypsin inhibitor, protects tissue from enzymes from inflammatory cells, especially elastase and is present in human blood. Disorders of the enzyme include human alpha-1 antitrypsin deficiency, a hereditary disorder in which lack of Human alpha-1 Antitrypsin AAT (A1AT) leads to uninhibited tissue breakdown during inflammation.
Studies indicate that this causes pulmonary emphysema and leads to liver cirrhosis in severe cases. Human alpha-1 antitrypsin protects the lungs from the harmful effects of human neutrophil elastase.
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
SERPINA1: 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. Defects in SERPINA1 are the cause of alpha-1-antitrypsin deficiency (A1ATD). A disorder whose most common manifestation is emphysema, which becomes evident by the third to fourth decade. A less common manifestation of the deficiency is liver disease, which occurs in children and adults, and may result in cirrhosis and liver failure. Environmental factors, particularly cigarette smoking, greatly increase the risk of emphysema at an earlier age. Belongs to the serpin family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted; Inhibitor; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 14q32.1
Cellular Component: Golgi apparatus; proteinaceous extracellular matrix; extracellular space; endoplasmic reticulum; extracellular region
Molecular Function: serine-type endopeptidase inhibitor activity; identical protein binding; protein binding; protease binding; glycoprotein binding
Biological Process: platelet activation; response to chromate; platelet degranulation; response to triglyceride; response to cytokine stimulus; response to lead ion; response to methanol; acute-phase response; response to hypoxia; response to lipopolysaccharide; blood coagulation; response to estradiol stimulus
Disease: Alpha-1-antitrypsin Deficiency; Pulmonary Disease, Chronic Obstructive
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Product Notes
The A1AT serpina1 (Catalog #AAA173006) is an Active Protein and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "Alpha-1-Antitrypsin, Active Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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