Home \ Antibodies \ Alpha-1-antitrypsin, Human, mAb 3C11
Catalog # HM2358

Alpha-1-antitrypsin, Human, mAb 3C11

125.001,174.00

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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.

Application
Immuno assays, Western blot

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.

Product type
Monoclonal antibodies
Amount
100 µg, 20 µg, 500 µg
Formulation
Quantities >500 µg will be send without preservative and carrier free. Quantities <500 µg will be send with preservative and carrier. exact formulation can be found on the datasheet.
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<br> - 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
CoA-TDS HM2300-HM2400
HM2358
84 kb
Safety Data Sheet
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