{"product_id":"cst-55573s","title":"CST,  55573S, SERPINA1 (F6I2Z) Rabbit Monoclonal Antibody","description":"Monoclonal Antibody for studying SERPINA1. Validated for Western Blotting,Simple Western™. Available in 2 sizes. Highly specific and rigorously validated in-house, SERPINA1 (F6I2Z) Rabbit Monoclonal Antibody (CST #55573) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nWestern Blotting: 1:1000\nSimple Western™: 1:10 - 1:50\n\u003cb\u003eStorage\u003c\/b\u003e\nSupplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg\/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.\n\u003cb\u003eProtocol\u003c\/b\u003e\nAvailable protocols: Western Blotting\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nSERPINA1 (F6I2Z) Rabbit Monoclonal Antibody recognizes endogenous levels of total SERPINA1 protein.\nSpecies Reactivity: Human\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nMonoclonal antibody is produced by immunizing animals with recombinant protein specific to human SERPINA1 protein.\n\u003cb\u003eBackground\u003c\/b\u003e\nAlpha-1-antitrypsin (SERPINA1), also known as AAT and PI, is a glycoprotein belonging to the serpin (serine protease inhibitor) family. The protein is mainly produced by hepatocytes and secreted into plasma, functioning as an inhibitor of cathepsin G and elastase released by neutrophil leukocytes (1). Alpha-1 antitrypsin deficiency (AATD) is one of the most common genetic diseases and is caused by mutations in the gene (2). The mutational variant inefficiently folds into its final conformation, thereby resulting in 85% retention as large protein polymers, which is toxic to the hepatocytes and leads to progressive liver disease, cystic fibrosis, and cirrhosis (3,4). In lung, AATD results in reduced levels of SERPINA1 and causes imbalance between elastase and anti-elastase activity. Long term AATD leads to progressive, irreversible destruction of lung tissue, and ultimately the development of chronic obstructive pulmonary disease with early-onset emphysema (5).\n\u003cb\u003eAlternate Names\u003c\/b\u003e\nA1A; A1AT; AAT; alpha-1 antitrypsin; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Alpha-1-antitrypsin; alpha-1-antitrypsin short transcript variant 1C4; alpha-1-antitrypsin short transcript variant 1C5; alpha1AT; epididymis secretory sperm binding protein; MGC23330; MGC9222; nNIF; PI; PI1; PRO2275; protease inhibitor 1 (anti-elastase), alpha-1-antitrypsin; serine (or cysteine) proteinase inhibitor, clade A, member 1; serine proteinase inhibitor, clade A, member 1; Serpin A1; serpin family A member 1; serpin peptidase inhibitor clade A (alpha-1antiproteinase, antitrypsin) member 1; serpin peptidase inhibitor clade A member 1; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1; SERPINA1; Short peptide from AAT; SPAAT\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: H\nSENSITIVITY: Endogenous\nMW (kDa): 62, 55\nSource\/Isotype: Rabbit IgG","brand":"CST","offers":[{"title":"Default Title","offer_id":46799818883241,"sku":"55573S","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-55573s","provider":"Iright","version":"1.0","type":"link"}