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

The cores of ferritins isolated from different organs of human subjects with beta-thalassemia/hemoglobin E (beta-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 t...

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Bibliographic Details
Published inBiology of metals Vol. 4; no. 3; p. 162
Main Authors St Pierre, T G, Tran, K C, Webb, J, Macey, D J, Heywood, B R, Sparks, N H, Wade, V J, Mann, S, Pootrakul, P
Format Journal Article
LanguageEnglish
Published Germany 01.01.1991
Subjects
Ferritins - chemistry
Ferritins - ultrastructure
Hemoglobin E - chemistry
Humans
Microscopy, Electron
Organ Specificity
Thalassemia - metabolism
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Summary:The cores of ferritins isolated from different organs of human subjects with beta-thalassemia/hemoglobin E (beta-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. beta-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 of beta-thal/HbE subjects were found to have larger, more crystalline cores than those from the beta-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.
ISSN:0933-5854
DOI:10.1007/BF01141308