Anti-Alpha 1 Antitrypsin / AAT / SERPINA1, clone AAT/4487

1 mg/ml in 1X PBS, BSA free, sodium azide free. Alpha-1 Proteinase Inhibitor antibody, also known as AAT antibody, recognizes a secreted serine protease inhibitor encoded by the SERPINA1 gene on chromosome 14q32.13. Alpha-1 Proteinase Inhibitor, commonly referred to as AAT and historically known as alpha-1 antitrypsin, is a major member of the clade A serpin family. It is synthesized primarily by hepatocytes and secreted into the circulation, where it functions as a critical regulator of proteolytic activity. Within tissues, Alpha-1 Proteinase Inhibitor is typically localized to the cytoplasm of hepatocytes and may also be detected in macrophages and certain epithelial cells under inflammatory conditions.Alpha-1 Proteinase Inhibitor plays an essential role in protecting tissues from excessive protease-mediated damage. Its principal physiologic target is neutrophil elastase, and inhibition of this enzyme preserves extracellular matrix integrity, particularly in the lung. AAT antibody is widely used in liver and pulmonary research to evaluate protein expression, identify hepatocellular differentiation, and assess inflammatory responses. Strong cytoplasmic staining in hepatocytes reflects active synthesis and secretion of this serpin.Structurally, Alpha-1 Proteinase Inhibitor contains a conserved reactive center loop that enables irreversible binding to target proteases. The protein is glycosylated and secreted as a stable circulating inhibitor. Genetic variants in SERPINA1 can lead to misfolding and intracellular polymerization of AAT within hepatocytes, resulting in reduced serum levels and accumulation of cytoplasmic inclusions. These alterations are associated with both pulmonary and hepatic pathology.Deficiency of Alpha-1 Proteinase Inhibitor is linked to early-onset emphysema, chronic obstructive pulmonary disease, and liver disorders including hepatitis and cirrhosis. Altered expression patterns may also be observed in inflammatory and neoplastic contexts. Clone AAT/4487 recognizes Alpha-1 Proteinase Inhibitor and is suitable for detecting AAT expression in relevant research applications.