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Alpha-1 Antitrypsin Deficiency

Alpha-1 Antitrypsin Deficiency (Alpha-1) is a genetic (inherited) condition – it is passed from parents to their children through their genes. Alpha-1 may result in serious lung disease in adults and/or liver disease at any age. Earlier screening helps to accurately diagnose Alpha-1, allowing for a treatment plan to be established.

Alpha-1 antitrypsin (A1AT) is a protein produced primarily by hepatocytes and released into circulation by the liver. A1AT functions in the lungs to inactivate neutrophil elastase, a powerful protease that contributes to the innate immune response.

A1AT keeps neutrophil elastase levels in check, thereby protecting the lung against alveolar wall destruction. A1AT provides > 90% of the anti-neutrophil elastase protection in the lungs.

Key Publications

Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomized, double-blind, placebo-controlled trial
Chapman KR, et al. Lancet. 2015
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Multi-Center Study: The Biochemical Efficacy, Safety and Tolerability of a New α1-Proteinase Inhibitor, Zemaira
Stocks J, et al. COPD. 2006
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