catalog number :
MBS844051
products full name :
Alpha 1 Antitrypsin, Human Plasma
products short name :
[Alpha 1 Antitrypsin]
products name syn :
[Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1; AAT; PI]
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
purity :
>=95% by SDS-PAGE
form :
Lyophilized from 30 mM Na Phosphate, pH 6.5 with 300 mM NaCl.
storage stability :
Store at -20 degree C or lower. Avoid repeated freezing and thawing cycles.
tested application :
SDS-PAGE
image1 heading :
Testing Data
other info1 :
Source: Human Plasma. Shown to be non-ractive for HBsAg, anti-HCV, anti-HBc, and negative for anti-HIV1&2 by FDA approved tests. Extinction Coefficient: 0.433. Activity: Shows 75-100% inhibition when tested with active-site titrated porcine pancreatic trypsin using Na-Benzoyl-L-Arginine-para-Nitroanilide Hydrochloride (L-BAPNA) as substrate.
products categories :
Proteins and Enzymes; Native Proteins (NEW CATEGORY)Proteins and Enzymes; Proteases; Trypsin (NEW CATEGORY)Proteins and Enzymes; Proteins and Enzymes (A-Z)Apoptosis & Related Products; Non-Caspase Proteases; Trypsin
products description :
Background: 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.
ncbi acc num :
NP_000286.3
ncbi gb acc num :
NM_000295.4
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 (1269340); Platelet Activation, Signaling And Aggregation Pathway (1269350); Platelet Degranulation Pathway (1269367); Response To Elevated Platelet Cytosolic Ca2+ Pathway (1269365)
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, signal peptide; Inhibitor; Secreted. 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