1 Introduction
2 Molecular mechanisms of body iron metabolism
Molecules for intestinal iron absorption
|
Divalent metal transporter 1 (DMT1) |
Duodenal cytochrome b (Dcytb) |
Heme carrier protein (HCP) |
Hemeoxygenase-1 |
Ferroportin |
Hephaestin |
Transferrin |
Molecules for bone marrow iron uptake
|
Transferrin receptor 1 |
Transferrin |
Molecules for reutilization of senescent red blood cells
|
Hemeoxygenase-1 |
Ferroportin |
Transferrin |
Molecules for hepatic iron storage
|
Ferritin |
Hemosiderin |
Transferrin |
Transferrin receptor 1 |
Transferrin receptor 2 |
Non-transferrin-bound iron |
HFE |
β2-microglobin |
Divalent metal transporter 1 |
ZIP14 |
Hemojuvelin |
Molecules for systemic iron regulation
|
Hepcidin |
(Unknown erythroid regulator?) |
2.1 Intestinal iron absorption
2.2 Red blood cell iron reutilization in the reticulo-endothelial system (RES) and iron load by blood transfusion
2.3 Iron uptake and utilization in liver
2.4 Bone marrow iron metabolism and erythropoiesis
2.5 Systemic regulation of body iron metabolism
3 Forms of iron in serum and tissue
3.1 Iron in plasma: Tf-bound iron and non-Tf-bound iron (NTBI)
3.2 Iron in tissue: tissue ferritin and labile iron pool (LIP)
3.3 Serum ferritin
4 Measurement of body iron stores: comparison with serum ferritin
4.1 Direct measurement
4.2 Physical measurement of body iron
5 Toxic effect of iron overload on organ function
5.1 Mechanism of iron toxicity
5.2 Iron overload syndrome
Hereditary hemochromatosis and related disorders
| |
Hereditary hemochromatosis | Type 1 |
HFE gene (6p21.3) mutation | |
Type 2 | |
Subtype A: hemojuvelin gene (1q21) mutation | |
Subtype B: hepcidin gene (19q13) mutation | |
Type 3 | |
Transferrin receptor 2 gene (7q22) mutation | |
Type 4 | |
Ferroportin gene (2q32) mutation | |
Ferritin gene mutation | H-ferritin gene mutation (mRNA iron-responsive-element mutation) |
DMT1 gene mutation | |
Ceruloplasmin gene mutation | |
Atransferrinemia | Transferrin gene mutation |
Secondary iron overload
| |
Ineffective erythropoiesis | Thalassemia, sideroblastic anemia, myelodysplastic syndromes |
Administration of iron for long periods | Take orally or intravenous injection |
Transfusion for long periods | |
Dietary iron overload | |
Liver dysfunction | Alcoholic liver injury, chronic hepatitis (type C), non-alcoholic steatohepatitis |
Others | Porphyria |