Reviewed by Karina A. Serban, MD, Robert A. Sandhaus, MD, PhD, FCCP
Alpha-1 antitrypsin protein is important in the protection of the lungs

Alpha-1 antitrypsin protein acts to protect the lungs’ air sacs, or alveoli, from damage when the immune system is doing work to protect the lungs. One type of the body’s own white blood cells, the neutrophil, releases a chemical called neutrophil elastase when it encounters inflammation or infection. Although neutrophil elastase can be helpful in protecting the lungs from attach, it can also damage the lungs and cause emphysema if not held in check.

Alpha-1 antitrypsin protein holds blocks elastase and protects normal tissue in the lungs. If there is not enough alpha-1 antitrypsin produced in and released by the liver, there will not be enough to protect the lungs’ alveoli, and emphysema or COPD develops. This is Alpha-1 lung disease.

About 1 in every 3,500 people has severe Alpha-1. The majority of people who have Alpha-1 are of Northern European decent, especially Scandinavians, but it has been found in all ethnic groups.


What specialist should I see if I have Alpha-1 lung disease?

If you have any abnormal Alpha-1 genes (e.g., Z, S, F, null), discuss the risk of lung disease with your Alpha-1 doctor. Different Alpha-1 genes lead to different risk for disease. You may be referred to a pulmonologist who specializes in Alpha-1 lung disease.


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