A1AT coats lungs, protecting them from neutrophil elastase.
Alpha-1 antitrypsin is made in the liver and released into the bloodstream where it travels to the lungs.
Neutrophil elastase is a powerful enzyme produced by white blood cells that helps break down harmful bacteria in the lung. Unchecked, neutrophil elastase can cause damage to lung tissue.
Lungs lack A1AT coating, leaving them open to damage by neutrophil elastase.
A1AT Misfolded A1AT trapped in the liver, causing liver damage
Neutrophil elastase Uninhibited, causing progressive lung damage
In this image, the squares inside the lungs represent the A1AT protein that normally binds neutrophil elastase, which is represented by the light blue circles.
Professional healthcare organizations such as the American Thoracic Society and the Medical and Scientific Advisory Committee of the Alpha-1 Foundation recommend Alpha-1 testing for everyone with the following risk factors6:
*Family testing of first-degree relatives is currently the most efficient way to detect Alpha-1.