Alpha-1-antitrypsin, Human, pAb

Catalog #: HP9063
Quantity: 100 µg
$412.00

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Description
Details
The goat polyclonal antibody recognizes human alpha-1-antitrypsin. This protein 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 HP9063
Product type Polyclonal antibodies
Quantity 100 µg
Formulation 0.2 µm filtered and protein G purified in PBS+0.1%BSA+0.02%NaN3
Immunogen Monomeric Z AAT purified from donor plasma
Isotype Goat Ig
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: Polyclonal antibody HP9063 can be used as detection antibody.
    - W: A reduced sample treatment and SDS-Page was used. The band size is ~60 kDa
  • Use:
    Dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions.
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