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Iron in hemochromatosis : deleterious effects of an essential nutrient

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Normal dietary iron causes organ damage in patients with homozygous Hereditary Hemochromatosis (HH). Heterozygotes (10% of the European population) are at risk for early cardiovascular death. HH patients absorb too much iron and have toxic non-transferring-bound iron (NTBI) in plasma. This project intends to identify the mechanism of TBI toxicity, and the damage to vascular endothelium (as target for arteriosclerosis) and to the liver. An inexpensive method for NTBI measurement will be developed. Oral iron chelator will be developed to inhibit excess absorption of iron and to scavenge NTBI. Iron absorption, as a key pathogenic mechanism, will be analysed at a molecular level. The project is expected to result in less organ damage and in prevention of early death in HH patients.

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