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Alpha-1 antitrypsin is a glycoprotein primarily produced by the liver and circulates in plasma at concentrations of 95–350 mg per 100 ml. As a member of the serpin superfamily, A1AT’s main function is to protect tissues, especially in the lungs, from proteolytic damage by inhibiting enzymes such as neutrophil elastase. By binding to and inactivating elastase, A1AT prevents the breakdown of elastin in connective tissue, thereby preserving lung structure and function.
Deficiency of A1AT, most often due to mutations in the SERPINA1 gene, leads to unregulated elastase activity. This can cause early-onset pulmonary emphysema and chronic obstructive pulmonary disease (COPD), as well as juvenile or adult liver cirrhosis due to accumulation of abnormal A1AT in the liver. Less commonly, deficiency can result in skin disorders like panniculitis.
Clinically, A1AT levels rise during inflammation, malignancy, liver disease, pregnancy, after surgery, and with oral contraceptive use. Measurement of A1AT, often alongside ceruloplasmin, aids in distinguishing different liver disorders. Therapeutically, purified A1AT is used for augmentation therapy in individuals with severe deficiency, and research continues into its broader anti-inflammatory and immunomodulatory applications.
Common uses include Proteolytic enzyme studies, assay standards, IVD standards, calibrators and controls.
Not a hazardous substance or mixture.
11 - Combustible Solids
