Hepcidin

• OMIM: 606 464
• UniProt: P81172

Hepcidin names of two proteins with 20 and 25 amino acids in higher mammals. Hepcidin -20 plays an important role in the regulation of iron metabolism by slows the absorption of iron through the intestine, the placenta and on the release of iron from the reticulo-endothelial system (RES). First hepcidin -20 has been described as a peptide, which is involved in microbial defense. Hence the name is derived hepatic bactericidal protein. A Hepcidinmangel is in autosomal recessive forms of hemochromatosis (type 2B) involved, which is caused by a mutation in the HFE gene.

Education

The body produces Hepcidin from a precursor, the pro- hepcidin in the liver. Pro- hepcidin is encoded by the gene HAMP (HFE - 2B). It is increasingly formed when iron and oxygen (at a good supply of iron and good transport oxygen present in the tissue) and interleukin -6 ( for inflammation ) are increasingly available.

Effects

Hepcidin -20 binds, for example, in Dünndarmmucosazellen and in macrophages of ferroportin, which is normally transported out iron from the cell interior. Is hepcidin bound to ferroportin, these cells can export no more iron and deliver to the transport protein transferrin in the blood. Dünndarmmucosazellen can before they are shed into the intestine, export their recorded over the ferroportin iron again only in the last two days; if a lot of ferroportin is inactivated by hepcidin, the iron absorbed into these cells goes with the Zellabschilferung back over the chair lost. So Hepcidin regulates down iron absorption in the intestine. Hepcidin also plays an important role in the alteration of the iron metabolism in the context of chronic inflammation. The increased inflammation in such interleukin-6 causes an increase of the Hepcidinspiegels. This then keeps the iron in the macrophages, which break down old red blood cells in the spleen, and prevents an instantly recycling, which then leads to an inflammation- induced anemia.

Mammals can not actively excrete iron. The iron balance is thus regulated mainly on iron uptake by the hepcidin.

A hepcidin agonist may be used by the inhibition of ferroportin - controlled iron transport from the intestinal cell in iron overload as in hemochromatosis or under secondary increased iron levels in the body, such as congenital anemias such as thalassemia or sideroblastic anemia. A first hepcidin agonist in the form of a synthetic protein that is similar to the first nine amino acids of hepcidin, but has thus far been modified so that it can be taken orally, proved to be a " Minihepcidin " in initial trials lowered, effectively cross the blood- iron concentration and prevented an iron accumulation in the liver. Similarly, a hepcidin antagonist for the treatment of anemia in the setting of chronic diseases, and in rare iron resistance pattern of iron deficiency anemia, which is an increased hepcidin formation based on in development.

Laboratory values

Hepcidin and its precursor, the pro- hepcidin can be detected in blood and urine.