Alpha-1-antitrypsin 0.1 mg
Source: Chicken
Purity: Immunoaffinity Purified
Clonality: Polyclonal
Crossreactivity: Human, mouse, rat
Format: Format: Phosphate-Buffered Saline. 0.01% Sodium Azide added as preservative.
Storage: 4 degree C for short term (weeks) and -20 degree C for long term. Avoid frequent freeze and thaw.
Stability: 6-12 months at -20 degree C.
Shipping: Products may be shipped on ice pack or dry ice. SERPINA1 ANTIBODY TARGET DESCRIPTION: Synonym Names for SERPINA1 antibody: SERPINA1; AAT; PI; A1A; PI1; A1AT; MGC9222; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serine (or cysteine) proteinase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1; Protease inhibitor (alpha-1-antitrypsin); protease inhibitor 1 (anti-elastase), alpha-1-antitrypsin
Function: Inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin.
Subcellular Location: Secreted protein.
Tissue Specificity: Plasma.
Domain: The reactive center loop (RCL) extends out from the body of the protein and directs binding to the target protease. The protease cleaves the serpin at the reactive site within the RCL, establishing a covalent linkage between the carboxyl group of the serpin reactive site and the serine hydroxyl of the protease. The resulting inactive serpin-protease complex is highly stable.
Polymorphism: The sequence shown is that of the M1V allele which is the most common form of PI (44 to 49%). Other frequent alleles are: M1A 20 to 23%; M2 10 to 11%; M3 14 to 19%.
Disease: The major physiological function of AAT is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE). A hereditary deficiency of AAT, is associated with a 20-30 fold increased risk of developing chronic obstructive pulmonary disease.
Disease: Deficiency of the normal inhibitor in individuals homozygous for allele Z or M-Malton can result in the development of chronic emphysema or infantile liver cirrhosis.
Disease: Variant Pittsburgh is the cause of bleeding diathesis.
Similarity: Belongs to the serpin family.
Alpha-1-antitrypsin reacts with human, mouse, rat.
OMIM: 107400; gene+phenotype. [NCBI / EBI]
Pathways: KEGG pathway: Complement and coagulation cascades 04610 Reactome Event:Hemostasis 109582
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