Alpha-1-antitrypsin

Introduction

Alpha-1-antitrypsin belongs to the protein structures, i.e. proteins that float in blood serum. The name comes from the study to identify these proteins. In serum white electrophoresis, these proteins are in the alpha-1 group.

Alpha-1-antitrypsin is an antagonist of trypsin, an enzyme that cleaves proteins. This trypsin, which is harmful in the blood, is therefore inhibited by alpha-1-antitrypsin. Since alpha-1-antitrypsin not only inhibits trypsin, but also other enzymes, it is also called a protease inhibitor.

Tasks, functions and benefits of alpha-1-antitrypsin

Alpha-1-antitrypsin is an inhibitor of protein-splitting enzymes. It belongs to the proteins of the Serpin family, which are encoded on the Serpin genes. In the digestive system, protein-splitting enzymes such as trypsin are important aids, while in the blood they are harmful.

In the blood, serum proteins that the body needs can be destroyed and this is prevented by Alpha-1-Antitrypsin. Serum proteins are a large group of different proteins, which can be divided into different subgroups in studies. Among other things, they have tasks in the immune defence and blood clotting of the body.

Most of these proteins are produced in the liver. Increased degradation by enzymes would have many harmful consequences for humans. Since it is not just trypsin, the more general name is protease inhibitor.

Besides alpha-1-antitrypsin, there are many other protease inhibitors. Proteases are also part of blood clotting and many other processes in the body. Protease inhibitors can also be used as drugs.

Certain thrombin inhibitors, i.e. substances that prevent blood clotting, can be used as heart attack prophylaxis. Protease inhibitors can also be used to treat some viral diseases. The amount of alpha-1-antitrypsin in the blood increases when inflammation occurs in the body.

During the acute phase reaction, increased alpha-1-antitrypsin is produced and can thus reduce an excessive immune reaction and the effect of neutrophil granulocytes, which would otherwise lead to the destruction of the body’s own elastin. A mutation in the serpine genes can lead to defective alpha-1-antitrypsin, which becomes enriched in the body and thus has many harmful consequences for the person affected. Furthermore, the deficiency of alpha-1-antitrypsin itself has many consequences, as blood clotting and the immune system develop disorders.

The genetic disease is particularly widespread in north-western Europe. So far, alpha-1-antitrypsin cannot be produced artificially. However, it can be extracted from the blood serum of healthy people and concentrated to help with a deficiency. Alpha-1-antitrypsin therefore has extensive functions in the human body and a deficiency has far-reaching consequences.

What are the alpha-1-antitrypsin norms?

Alpha-1-antitrypsin can be determined in the blood. The normal range is between 83 and 199 milligrams per deciliter. An increase need not be pathological, but can also occur during pregnancy.

A normal range is always a pure statistic. Not every person whose values are different is automatically ill. Some people have different values throughout their lives and never show symptoms.

Such a laboratory value can always only serve as a supporting diagnosis and not as the sole basis for a diagnosis. A rapid test can be used to detect an abnormal variant of alpha-1-antitrypsin. This variant cannot perform its normal function and thus effectively leads to alpha-1-antitrypsin deficiency.

The test works with a simple blood sample from the fingertip and the result is available within minutes. However, the test can only rule out the presence of this defective variant and not the general deficiency disease. Alpha-1-antitrypsin is produced in the human liver and then leaves the liver via the blood.

Alpha-1-antitrypsin is encoded on the genes of the serpine group on the fourteenth chromosome. The genes are read in the liver cells and translated into amino acids by the ribosomes. The amino acid chain must then be folded appropriately to form alpha-1-antitrypsin. During inflammatory reactions the liver cells become more active and the production of many substances, including alpha-1-antitrypsin, is increased.