catalog number :
MBS850058
products full name :
AAT Antibody
products short name :
AAT
products name syn :
PI; A1A; AAT; PI1; A1AT; SERPINA1
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 A1Cleaved into the following chain:Short peptide from AAT; SPAAT
other gene names :
SERPINA1; SERPINA1; PI; A1A; AAT; PI1; A1AT; PRO2275; alpha1AT; AAT; PI; SPAAT
uniprot entry name :
A1AT_HUMAN
reactivity :
Human, Mouse
form :
Ascitic fluid containing 0.03% sodium azide.
concentration :
100ug/100ul
storage stability :
Store at 4 degree C. For long term storage, store at -20 degree C
tested application :
Western Blot (WB), ELISA (EIA)
app notes :
WB: 1/500 - 1/2000, ELISA: Propose dilution 1/10000. Not yet tested in other applications. Determining optimal working dilutions by titration test.
other info2 :
Immunogen: Purified recombinant fragment of human AAT expressed in E. Coli. Species: Mouse IgG1
products categories :
Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells; Autophagy antibody
products description :
Background: AAT, also known as SERPINA1; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1. It is secreted and is a serine protease inhibitor whose targets include elastase, plasmin, thrombin, trypsin, chymotrypsin, and plasminogen activator. Severe Serpin A1 deficiency leads to several clinical complications such as pulmonary emphysema, juvenile hepatisis, cirrhosis and hepatocellular carcinoma.
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: Secreted; Secreted, signal peptide; Inhibitor. 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; response to hypoxia; acute-phase response; response to lipopolysaccharide; blood coagulation; response to estradiol stimulus. Disease: Alpha-1-antitrypsin Deficiency; Pulmonary Disease, Chronic Obstructive