Hemin Augments Growth and Hemoglobinization of Erythroid Precursors from Patients with Diamond-Blackfan AnemiaReportar como inadecuado




Hemin Augments Growth and Hemoglobinization of Erythroid Precursors from Patients with Diamond-Blackfan Anemia - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

AnemiaVolume 2012 2012, Article ID 940260, 4 pages

Research Article

Department of Hematology, Hadassah – Hebrew University Medical Center, Ein-Kerem, Jerusalem 91120, Israel

Departments of Pediatrics and Bone Marrow Transplantation, Hadassah – Hebrew University Medical Center, Jerusalem 91120, Israel

Received 19 November 2011; Revised 19 February 2012; Accepted 4 March 2012

Academic Editor: Aurelio Maggio

Copyright © 2012 Eitan Fibach and Memet Aker. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Diamond-Blackfan anemia DBA is congenital pure red-cell anemia due to a differentiation block in erythroid precursors. The disease is commonly caused by mutations in genes for ribosomal proteins. Despite the identification of disease causal genes, the disease pathogenesis is not completely elucidated. The ribosomal abnormalities are assumed to inhibit globin translation which may lead to excess free heme, stimulating a generation of free radicals and thereby damaging the precursors. We studied the effect of hemin heme chloride on cultured human erythroid precursors and found that contrary to aforementioned hypothesis, although hemin moderately stimulated free radicals, it did not cause apoptosis or necrosis. In erythroid precursors derived from DBA patients, hemin significantly stimulated growth and hemoglobinization. Thus, heme toxicity is unlikely to play a role in the pathophysiology of most DBA cases. Moreover, its beneficial effect in culture suggests a therapeutic potential.





Autor: Eitan Fibach and Memet Aker

Fuente: https://www.hindawi.com/



DESCARGAR PDF




Documentos relacionados