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Iron Overload in Aceruloplasminemia

In aceruloplasminemia, a deficiency in ceruloplasmin (also known as ferroxidase) prevents adequate oxidization of Fe²⁺ to Fe³⁺. As transferrin only binds iron in the ferric state, this inhibits the binding of iron by transferrin,(1,2) causing the absorption of NTBI from the diet, and exposing vital organs to oxidative stress. The movement of iron from intracellular stores into plasma is impaired, leading to iron overload.