Alpha-1-antitrypsin Recombinant Rabbit mAb (SDT-943-127)

Alpha-1-antitrypsin (AAT) is a 52 kDa acute-phase glycoprotein synthesized primarily by hepatocytes and secreted into plasma, where it acts as the principal serine protease inhibitor (serpin) by irreversibly binding and neutralizing neutrophil elastase, proteinase 3 and cathepsin G to protect lung and other connective tissues from proteolytic damage, while its deficiency due to PIZ or PIS mutations leads to misfolded AAT accumulation in hepatocytes causing neonatal hepatitis or cirrhosis and reduced circulating levels that leave pulmonary elastin unopposed and predispose to early-onset panlobular emphysema, intravenous augmentation therapy with purified plasma or recombinant AAT being the standard treatment aimed at maintaining serum levels above 11 μM for lung protection.