catalog number :
MBS602022
products full name :
a1-Antitrypsin (AAT, alpha-1-AT, alpha1 Proteinase Inhibitor, alpha1 PI)
products short name :
a1-Antitrypsin
products name syn :
Anti -a1-Antitrypsin (AAT, alpha-1-AT, alpha1 Proteinase Inhibitor, alpha1 PI)
other names :
alpha-1-antitrypsin; Alpha-1-antitrypsin; alpha-1-antitrypsin; serpin A1; alpha-1-antiproteinase; alpha-1-antitrypsin null; alpha-1 protease inhibitor; protease inhibitor 1 (anti-elastase), alpha-1-antitrypsin; serine (or cysteine) proteinase inhibitor, clade A, member 1; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1
other gene names :
SERPINA1; SERPINA1; PI; A1A; AAT; PI1; A1AT; PRO2275; alpha1AT; AAT; PI; SPAAT
uniprot entry name :
A1AT_HUMAN
specificity :
Recognizes human Alpha-1-Antitrypsin (AAT). No crossreactivity with alpha-1-acid glycoprotein, serum albumin or other serum proteins.
purity :
Affinity Purified. Purified by Protein A affinity chromatography.
form :
Supplied as a liquid in PBS, pH 7.4, 0.9% sodium azide.
concentration :
2.76mg/ml
storage stability :
May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
tested application :
ELISA (EL/EIA)
app notes :
Suitable for use in ELISA.
other info1 :
Immunogen: Purified human Alpha-1-Antitrypsin (AAT)
other info2 :
Hybridoma: P3X63-Ag8.653 myeloma cells with spleen cells from Balb/c mice.
products categories :
Antibodies; Abs to Coagulation Factors
products description :
Alpha-1-antitrypsin (AAT) consisting of a single polypeptide chain and has a molecular weight of 51kD. It exists in a number of genetic variants. MM variant is the most common. AAT is synthesized in the liver and it acts as an inhibitor of proteases such as trypsin, elastase, chymotrypsin, collagenase, leukocytic proteases, plasmin and thrombin, which may be released during inflammatory reactions in the lung. In the absence of AAT, these enzymes are not inhibited and they may digest pulmonary parenchyma. AAT deficiency is associated with chronic obstructive lung disease (emphysema) and less frequently with hepatic cirrhosis in infants and respiratory distress of the newborn. Increase in AAT occurs as an acute phase response to tissue necrosis and inflammation. Serum level of AAT is elevated in rheumatoid arthritis, bacterial infections, vasculitis and carcinomatosis. In colorectal and lung carcinomas, AAT has also related to their invasive and metastatic capacity. The average level of AAT in plasma is 1.3g/L.
ncbi acc num :
NP_001121179.1
ncbi gb acc num :
NM_001127707.1
ncbi mol weight :
46,737 Da
ncbi pathways :
Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); FOXA1 Transcription Factor Network Pathway (137979); Hemostasis Pathway (106028); Platelet Activation, Signaling And Aggregation Pathway (106034); Platelet Degranulation Pathway (106050); Response To Elevated Platelet Cytosolic Ca2+ Pathway (106048)
ncbi summary :
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 summary :
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; Secreted, signal peptide; Inhibitor. Chromosomal Location of Human Ortholog: 14q32.1. Cellular Component: Golgi apparatus; extracellular space; proteinaceous extracellular matrix; 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; acute-phase response; response to hypoxia; response to methanol; response to lipopolysaccharide; blood coagulation; response to estradiol stimulus. Disease: Alpha-1-antitrypsin Deficiency; Pulmonary Disease, Chronic Obstructive