Porphyria, Erythropoietic
Using isolated duodenal loops to measure the transepithelial iron transport, we found that CEP mice presented a higher rate of iron absorption than the WT mice, although the differences between the area under the curves did not reach statistical significance (Figure 3D). PubMed:28143953
Serum iron was also increased in CEP mice, but this did not lead to elevated Tf saturation because Tf was also significantly increased, which is reminiscent of iron deficiency anemia and facilitates iron delivery to a larger number of erythroblasts (Table 1 and Figure 3G). PubMed:28143953
Iron was also detected in the macrophages of the red pulp of CEP mice, while almost no iron deposit was observed in the spleen of Hjv–/– mice (Figure 4B), confirming that Hb and “free” heme are the likely source of macrophage iron accumulation. PubMed:28143953
As expected, Perl’s staining of CEP kidneys showed significant accumulation of iron in the renal cortical part, particularly in the proximal tubules (Figure 6A). PubMed:28143953
Iron was also detected in the macrophages of the red pulp of CEP mice, while almost no iron deposit was observed in the spleen of Hjv–/– mice (Figure 4B), confirming that Hb and “free” heme are the likely source of macrophage iron accumulation. PubMed:28143953
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