What is the measurement of all iron bound to tranferrin in the body?
Serum iron (SI)
How does iron enter cells to be incorporated into heme?
Transferrin receptor binds 2 transferrins. The receptor-tranferrin-iron complex is endocytosed. The endocytotic vacuole fuses with a lysozyme, where at an acid pH, tranferrin releases iron as ferrous form. The iron is transported to mitochondria.
How does high plasma iron affect hepcidin synthesis and iron homeostasis?
Increases synthesis of hepcidin, which decreases release of iron from enterocytes and macrophages.
What genetic mutation is associated with Hemochromatosis Type 2A?
HJV gene that codes for hemojuvelin
What genetic mutation is associated with Hemochromatosis Type 4?
SLC40A1 gene that codes for Ferroportin
Iron study laboratory results of anemia of chronic anemia
Low serum iron, normal or low transferrin, low transferrin saturation, and high serum ferritin
How does low plasma iron affect hepcidin synthesis and iron homeostasis?
Decreases synthesis of hepcidin, which allows increased release of iron into circulation.
What is the biochemical significance of microcytic anemia?
Inadequate synthesis of hemoglobin
Describe the synthesis of [Fe-S] clusters
Begins in the mitochondria with elemental sulphur, the cofactor pryridoxal-5-phosphate and a scaffold protein. 2 iron atoms are added to the [Fe-S] cluster and form a single [2Fe-2S] cluster. 2 [2Fe-2S] clusters form a [4Fe-4S]