The new iron age (Review)

In the last four years there has been a major change in the approach to dia gnosis of the iron overload disorder hereditary haemochromatosis (HH) follo wing the discovery of the gene that is mutated in HH called HFE. In the fir st part of this review we will give a concise overview of the disease. Also the current literature on the role of HFE in iron absorption and transport at a molecular level and how mutations in HFE may lead to the break down i n the regulation of iron homeostasis is reviewed. The second part of the re view focuses on the molecular aspects of iron storage. Different chemical f orms of storage iron deposits such as ferrihydrite and geotite are present in the iron storage proteins ferritin and haemosiderin. The type of iron st orage deposits is thought to be an important factor in determining the degr ee of iron toxicity and tissue damage in patients with iron overload. Varia tions in the form of iron deposits in different types of iron overload dise ase e.g. HH or beta -thalessemia, the site of iron deposition and the clini cal treatment used will be discussed.