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Hereditary Hemochromatosis

An Algorithmic Approach to Diagnosis



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Effect of Hemochromatosis on Duodenal Crypt Cell

Slide 12

January 2009

Now let’s focus attention back to the duodenal crypt cell and the effect of reduced hepcidin synthesized by the liver. In the absence of hepciden, the ferroportin transporter is fully activated. And, like the hepatocyte, the membrane bound HFE protein in the crypt cell is modified which interferes with the formation of the beta‑2 microglobulin, HFE Protein,  transferrin receptor-1 complex. But, iron is still taken up from the blood capillary by the transferrin receptor-2 process which facilitates uptake and storage of iron in the crypt cell. Because of the complete activation of ferroportin, iron is continuously recirculated into the blood capillary where it is oxidized by hephaestin to ferric iron to circulate as highly saturated transferrin. One of the early findings in hemochromatosis is increased plasma transferrin and increased transferrin bound iron.

Effect of Hemochromatosis

 


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