Alpha-1-Antitrypsin Deficiency | Trypsinogen

Alpha-1-Antitrypsin Deficiency

The cause of alpha-1-antitrypsin deficiency is often a genetic defect. Alpha-1-antitrypsin is an enzyme that inhibits other enzymes in their function. The enzymes that are inhibited normally have the task of breaking down proteins, which causes them to lose their function.

Alpha-1-antitrypsin can therefore also be called a proteinase inhibitor. The enzymes that are inhibited by alpha-1-antitrypsin occur mainly in inflammatory processes and are mainly chymotrypsin, trypsin, plasmin, the elastase and thrombin. The inhibition of elastase is of particular importance here.

Elastase usually cleaves elastin, which is mainly found in the lungs. Elastin is a structural protein that is largely responsible for the elasticity of the lungs. A deficient presence of elastase inhibitors such as alpha-1-antitrypsin can lead to increased activity of elastase in the lungs.

Here, as everywhere else in the body, elastase breaks down proteins, but this affects the body’s own tissue in the lungs. This leads to massive damage to the lung tissue, which inevitably leads to a reduction in lung function. Symptoms that develop from this include coughing, shortness of breath and narrowing of the airways. In addition, the liver, which is mainly responsible for the formation of elastase, may show increased liver values and be further damaged by bile stasis.