|The SERPINA1 serpina1 (Catalog #MBS584815) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Alpha-1-antitrypsin, Human, mAb 2C1, Immunoassay Antibody reacts with Human and may cross-react with other species as described in the data sheet. MyBioSource\'s Alpha-1-antitrypsin can be used in a range of immunoassay formats including, but not limited to, Immunoassay, Immunofluorescence (IF), Immunoprecipitation (IP), Immunohistochemistry (IHC) Paraffin, Western Blot (WB).
The typical starting working dilution is 1:50. Researchers should empirically determine the suitability of the SERPINA1 serpina1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process.
The SERPINA1 serpina1 product has the following accession number(s) (GI #189163542) (NCBI Accession #NP_001121179.1) (Uniprot Accession #P01009). Researchers may be interested in using Bioinformatics databases such as those available at The National Center for Biotechnology Information (NCBI) website for more information about accession numbers and the proteins they represent. Even researchers unfamiliar with bioinformatics databases will find the NCBI databases to be quite user friendly and useful.
To buy or view more detailed product information and pricing, please click on the technical datasheet page below:
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 alpha1-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.
In general, we may offer more than one antibody to a given target to enable options for the researcher. Available antibodies recognizing SERPINA1 are readily searchable from our website. Different antibodies against the same target such as SERPINA1 may be optimized or tested for different applications and species. This enables researchers to select the option that may be best for their model system, to screen more than antibody to determine which one may be best for their model system, as well as to use more than one antibody to follow up on and validate their results. The following patways have been known to be associated with this gene.