catalog number :
MBS238024
products full name :
NATIVE HUMAN ALPHA 1 ANTITRYPSIN
products short name :
ALPHA 1 ANTITRYPSIN
other names :
alpha-1-antitrypsin; Alpha-1-antitrypsin; alpha-1-antitrypsin; alpha-1 protease inhibitor; alpha-1-antiproteinase; alpha-1-antitrypsin null; protease inhibitor 1 (anti-elastase), alpha-1-antitrypsin; serine (or cysteine) proteinase inhibitor, clade A, member 1; serpin A1; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1Short peptide from AAT; SPAAT
other gene names :
SERPINA1; SERPINA1; PI; A1A; AAT; PI1; A1AT; PRO2275; alpha1AT; AAT; PI; SPAAT
uniprot entry name :
A1AT_HUMAN
purity :
SDS PAGE: >96%. Identity was confirmed by radial immunodiffusion, SDS-PAGE and cellulose acetate electrophoresis.
form :
Purified. Purified alpha-1 antitrypsin from human serum - liquid
concentration :
Approximate Protein Concentration: 2 mg/ml
storage stability :
Store at -20 degree C only. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the protein. Should this product contain a precipitate we recommend microcentrifugation before use. Shelf Life: 18 months from date of despatch.
tested application :
ELISA (EIA)
other info2 :
Buffer Solution: Phosphate buffered saline. Target Species: Human
products description :
Alpha-1-antitrypsin (A1AT) is a glycoprotein of MW 54 kDa with a pI of 4.8. It is a serine protease inhibitor that acts to limit the amount of cell damage and death at the tissue site. Different isoenzymes exist which together form the Protease inhibitor system. Phenotype M is the most common and certain variants (S and Z) are associated with A1AT deficiency which can lead to emphysema and liver disease.
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 (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, signal peptide; 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 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