Iron is a major component of the Earth’s crust, but its own chemistry greatly limits utilization and also sets the basis for its toxicity. Hereditary hemochromatosis (HH) is the most common cause of iron overload in humans. For much of the twentieth century, HH was regarded as a monogenic disorder characterized by excess tissue deposits of iron inevitably producing organ damage. This view has been shattered by the identification of similar phenotypes associated with mutations of at least four different ironmetabolism genes (HFE, TfR2, HAMP, HJV) and the increasing appreciation of the disease’s multifactorial nature.
Hereditary hemochromatosis / F., Ferrara; Corradini, Elena; Pietrangelo, Antonello. - STAMPA. - Section VI:(2006), pp. 567-572. [10.1007/978-1-59259-963-9_54]
Hereditary hemochromatosis.
CORRADINI, Elena;PIETRANGELO, Antonello
2006
Abstract
Iron is a major component of the Earth’s crust, but its own chemistry greatly limits utilization and also sets the basis for its toxicity. Hereditary hemochromatosis (HH) is the most common cause of iron overload in humans. For much of the twentieth century, HH was regarded as a monogenic disorder characterized by excess tissue deposits of iron inevitably producing organ damage. This view has been shattered by the identification of similar phenotypes associated with mutations of at least four different ironmetabolism genes (HFE, TfR2, HAMP, HJV) and the increasing appreciation of the disease’s multifactorial nature.
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