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Organ-specific crystalline structures of ferritin cores in β-thalassemia/hemoglobin E
journal contribution
posted on 2023-05-18, 14:15 authored by St Pierre, TG, Tran, KC, Webb, J, Macey, DJ, Heywood, BR, Sparks, NH, Wade, VJ, Manna, S, Pootrakul, PThe cores of ferritins isolated from different organs of human subjects with β-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 of β-thal/HbE subjects were found to have larger, more crystalline cores than those from the β-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.
History
Publication title
Biology of MetalsVolume
4Pagination
162-165ISSN
0933-5854Publisher
Springer VerlagPlace of publication
NetherlandsRights statement
© Springer-Verlag 1991Repository Status
- Restricted