Home \ Antibodies \ Alpha-1-antitrypsin, Human, mAb 2C1
Catalog # HM2289

Alpha-1-antitrypsin, Human, mAb 2C1

120,001.283,00

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The mouse monoclonal antibody clone 2C1 recognizes polymeric forms of human alpha-1-antitrypsin. Alpha-1-antitrypsin is the most abundant circulating protease inhibitor. Serpinopathies are conformational diseases characterized by the polymerization and intracellular retention of members of the serine protease inhibitor or serpin superfamily of proteins.1 The best known is a1-antitrypsin deficiency, with the most common severe deficiency allele being the Z mutation (Glu342Lys). The severe Z deficiency allele (Glu342Lys) causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. This causes neonatal hepatitis, cirrhosis, and hepatocellular carcinoma.
Clone 2C1 recognizes polymers formed by Z α1-antitrypsin in vivo. It also recognizes polymers formed by the Siiyama (Ser53Phe) and Brescia (Gly225Arg) mutants, and the novel His334Asp shutter domain mutant of a1-antitrypsin that is associated with prolonged neonatal jaundice in a 6-week-old boy. These data show that Z and shutter domain mutants form polymers with a shared epitope.

Application
Immuno assays, Immuno fluorescence, Immuno precipitation, Paraffin sections, Western blot

Application Notes
W: a non-denaturating gel was used. The bands obtained were ~110, 165, 220 and 275 kDa.
IA: HM2289 can be used as capture and detection antibody.

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.

Positive Control
Human tonsil and kidney

Negative Control
For Western blot, any irrelevant protein can be used.

Product type
Monoclonal antibodies
Quantity
20 µg, 100 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
Z a1-antitrypsin polymers prepared from a1-antitrypsin purified from the plasma of PI*Z homozygotes
Isotype
Mouse IgG1
Species
Human
Alias
Alpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1, A1AT </br> - Gene name: SERPINA1
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

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