catalog number :
MBS534535
products full name :
alpha 1 Antitrypsin antibody
products short name :
alpha 1 Antitrypsin
products name syn :
Polyclonal alpha 1 Antitrypsin; Anti-alpha 1 Antitrypsin; A1AT; alpha 1; alpha-1 Antitrypsin; A1-AT
other names :
alpha-1-antitrypsin; Alpha-1-antitrypsin; alpha-1-antitrypsin; 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
reactivity :
Baboon, Horse, Mink, Monkey
form :
Purified antibody fraction from rabbit serum supplied in 10mM PBS, pH 7.4, with 0.2% BSA and 0.09% NaN3.
concentration :
1.0 ml per vial
storage stability :
Store at 4 degree C.
tested application :
Immunohistochemistry (IHC) Formalin/Paraffin
app notes :
IHC-F: 1:100-1:200. IHC-P: 1:100-1:200
other info1 :
Biological Significance: Alpha-1-antitrypsin (alpha-1-AT) exists in a number of genetic variants. MM variant is the most common. Alpha-1-AT is synthesized in the liver and it acts as an inhibitor of proteases such as trypsin, elastase, chymotrypsin, collagenase, leucocytic proteases, plasmin, and thrombin, which may be released during inflammatory reactions in the lung. In the absence of alpha-1-AT, these enzymes are not inhibited and they may digest pulmonary parenchyma. Alpha-1-AT 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 alpha-1-AT occurs as an acute phase response to tissue necrosis and inflammation. Serum level of alpha-1-AT is elevated in rheumatoid arthritis, bacterial infections, vasculitis, and carcinomatosis.
other info2 :
Biohazard Information: This product contains sodium azide as preservative. Although the amount of sodium azide is very small appropriate care must be taken when handling. Immunogen: Alpha 1 antitrypsin antibody was raised in rabbit using purified human serum Alpha-1-AT as the immunogen.
products categories :
Proteases, Inhibitors, & Enzymes
products description :
Rabbit polyclonal alpha 1 Antitrypsin antibody
ncbi acc num :
AAB59495.1
ncbi mol weight :
34,755 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: Inhibitor; Secreted; 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 hypoxia; acute-phase response; response to methanol; response to lipopolysaccharide; blood coagulation; response to estradiol stimulus. Disease: Alpha-1-antitrypsin Deficiency; Pulmonary Disease, Chronic Obstructive