The importance of Bmp6 and hemojuvelin in hepcidin expression is evident from the fact that disruption of mouse Bmp6[6], [7] or Hfe2[11], [12] genes results in severe hemochromatosis, which is in terms of tissue iron overload similar to hemochromatosis caused by disruption of the hepcidin gene itself. Here, HJV is linked to hemochromatosis type 1.