Alpha-1-antitrypsin (SERPINA1, AAT)

Size

£991.00

SKU:: 353400
Purity:: Purified by Protein G affinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: Z a1-antitrypsin polymers prepared from a1-antitrypsin purified from the plasma of PI*Z homozygotes
Clone:: 2C1
Format:: Supplied as a liquid in PBS, 0.1% BSA, 0.02% sodium azide.
Extra Details:: 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. 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. 353400 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. Applications: Suitable for use in ELISA, Western Blot, Immunoprecipitation, Immunofluorescence and Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 1:50. A non-reduced sample treatment and SDS-Page was used. Bands at ~110, 165, 220 and 275 kD. ELISA: 1:50, can be used as capture and detection antibody. Immunohistochemistry (paraffin): 1:50 Optimal dilutions to be determined by the researcher. Positive Control: Human tonsil and kidney Negative Control: For Western blot, any irrelevant protein can be used Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Shipping Conditions:: Blue Ice