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Alpha-1 antitrypsin, Human, mAb 3C11

Antibody clone 3C11 recognizes both the healthy monomeric form and the disease associated polymeric forms of human alpha-1-antitrypsin with equal affinity.
Catalog #: HM2358
Quantity: 100 µg
$474.00

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Description
Details
Antibody clone 3C11 recognizes both the healthy monomeric form (M variant) and the disease associated polymeric forms (Z variants) of human alpha-1-antitrypsin with equal affinity. Alpha-1-antitrypsin is a member of the serine protease inhibitor (serpin) superfamily which are proteins known for their ability to inhibit proteases. It is the most abundant circulating protease inhibitor known. It mainly targets enzymes released by neutrophils, especially neutrophil elastase (NE) but also proteinase 3 (PR3) and Cathepsin G (CG). Serpinopathies are conformational diseases characterized by the polymerization and intracellular retention of members of the serpin superfamily. The best known is alpha-1 antitrypsin deficiency, with the most common severe deficiency allele being the Z mutation (Glu342Lys). This severe autosomal dominant disorder causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. Due to this accumulation of polymers in hepatocytes, blood alpha-1 trypsin levels will decrease leading to chronic uninhibited tissue breakdown. This causes the degradation especially of lung tissue which will eventually lead to pulmonary emphysema. In addition, accumulation of polymers in hepatocytes causes liver diseases such as neonatal hepatitis, cirrhosis, and hepatocellular carcinoma.
Specifications

Specifications

Catalog number HM2358
Product type Monoclonal antibodies
Quantity 100 µg
Formulation 1 ml (100 μg/ml) 0.2 μm protein G purified antibody solution in PBS, containing 0.1% bovine serum albumin.
Immunogen Monomeric Z a1-antitrypsin purified from the plasma of a PI*ZZ homozygote (Ref.1)
Isotype Mouse IgG2a
Species Human
Alias Alpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1
Gene name: SERPINA1, AAT, PI
Storage and stability Product should be stored at 4°C. Under recommended storage conditions, product is stable for at least one year.
Precautions For research use only. Not for use in or on humans or animals or for diagnostics. It is the responsibility of the user to comply with all local/state and federal rules in the use of this product. Hycult Biotech is not responsible for any patent infringements that might result from the use or derivation of this product.
Disease Autoimmunity, Pulmonology
Application
Applications
  • Application:
    IA, W
  • Application Notes:
    IA: When used as a detection antibody in sandwich ELISA, the antibody 3C11 reacts with similar affinity with monomeric and polymeric alpha-1 antitrypsin (Ref.1).
    W: Non-denaturing (3–12% NativePAGE Bis–Trisgel) and SDS-PAGE gels were used to separate the samples. 6 M urea gel was prepared with 8% acrylamide (w/v) and 0.375 M Tris buffer (Ref.2). Both reduced and non-reduced sample treatment can be used.
  • Use:
    Dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.
References
References
References:
1. Ordoñez, A et al; A single-chain variable fragment intrabody prevents intracellular polymerization of Z a1-antitrypsin while allowing its antiproteinase activity. The FASEB Journal 2015, 29:2667
2. Tan, L et al; Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody. Int J Biochem Cell Biol 2015, 58:81
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