Organ-specific crystalline structures of ferritin cores in?-thalassemia/hemoglobin E

Abstract

The cores of ferritins isolated from different organs of human subjects withβ-thalassemia/hemoglobin E (β-thal/HbE) disease have different size distributions and crystallinities depending on the source organ. These patients have not been treated by hypertransfusion regimen or iron chelation therapy.β-Thal/HbE spleens and livers yield ferritin cores which are less crystalline than those isolated from normal spleens and livers, reflecting the more rapid deposition of iron in the diseased state. Ferritins isolated from the hearts and pancreases ofβ-thal/HbE subjects were found to have larger, more crystalline cores than those from theβ-thal/HbE livers and spleens, possibly as a consequence of the role of the heart and pancreas as long-term iron deposition sites in this iron overload pathology.