Apha-1 antitrypsin, or A1AT, is a type of serine protease inhibitor that helps protect the lung tissues from the damaging effects of excessive protease enzymes. The activity of protease enzymes, particularly neutrophil elastase, increases rapidly in the event of an inflammation or infection. Lack of A1AT causes these overactive enzymes to corrode alveolar walls and small air pathways in the lungs, which can lead to acute respiratory disorders.
A1AT is the most prominent serine protease inhibitor that inactivates enzymes by forming covalent bonds with them. A deficiency of A1AT is linked to hereditary conditions that can make the body vulnerable to diseases such as chronic obstructive pulmonary disease, emphysema and liver problems.
A1AT is primarily produced in the liver. Genetic defects can cause alterations in the structure of the A1AT molecule. As a result, the deformed A1AT molecule cannot pass out of the liver and enter into the bloodstream. This leads to acute deficiency of A1AT in the lungs and other parts of the body.
The accumulation of A1AT may trigger cell destruction in the liver, making it prone to damaging conditions such as cirrhosis. In addition to genetic conditions, environmental factors play an important role in the onset and extent of the effects of A1AT deficiency.