Jurnal Internasional Defisiensi Ferroportin dalam sel eritroid menyebabkan defisiensi besi serum dan meningkatkan hemolisis karena stres oksidatif
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Ferroportin (FPN), the only known exporter of iron in vertebrates, transports iron from the intestines, spleen and liver cells into the blood to deliver iron to tissues and other cells in vivo. Most of the iron circulating is consumed by erythroid cells to synthesize hemoglobin. Here we find that erythroid cells not only consume large amounts of iron, but also return large amounts of iron to the blood. Erythroblast-specific Fpn KO ( Fpn KO) mice developed lower serum iron levels in conjunction with excessive iron tissue and increased expression of FPN in the spleen and liver without changing hepcidin levels. Our results also show that Fpn. KO KOs, who suffer from mild hemolytic anemia, are sensitive to phenylhydrazine-induced oxidative stress but are able to tolerate iron deficiency after being exposed to low iron diets and phlebotomy, supporting anemia  FPN KO mice resulting from red erythrocytic iron and resulting oxidative injury rather than red blood cell (RBC) production defects. In addition, we found that the average corpuscular volume (MCV) of gain-of-function [FPN194522] patient mutations was positively associated with serum transferrin saturation, whereas MCV from loss-function FPN no mutation patients, supporting that erythroblasts donate iron to the blood via FPN in response to serum iron levels. Our results show that erythroid FPN cells play an unexpectedly important role in maintaining systemic iron homeostasis and protecting red blood cells from oxidative stress, providing insight into the pathophysiology of FPN disease.
- Submitted April 6 2018.  Received September 6, 2018.
- © 2018 by The American Society of Hematology