Citations to this article

Determination of the hemoglobin F program in human progenitor-derived erythroid cells.
A D Friedman, … , J Javid, D G Nathan
A D Friedman, … , J Javid, D G Nathan
Published April 1, 1985
Citation Information: J Clin Invest. 1985;75(4):1359-1368. https://doi.org/10.1172/JCI111837.
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Determination of the hemoglobin F program in human progenitor-derived erythroid cells.

Abstract

The absolute adult and fetal hemoglobin (HbF) contents of the erythroid cells derived from the differentiation of normal human and simian erythroid progenitors and of the peripheral blood erythroid burst-forming units (BFU-E) of patients with nondeletion hemoglobinopathies have been measured with a sensitive radioligand immunoassay. The HbF content varied between 0.13 and 2.96 pg/cell, representing between 0.7% and 19.6% of the total hemoglobin with a mean value of 7.0%. The absolute content of HbF was indistinguishable in the well-hemoglobinized progeny of marrow erythroid colony-forming units, marrow or blood BFU-E, or of mixed colony-forming units. The term HbF program refers to this inherent capacity to produce fetal hemoglobin (HbF) in the erythroid cells derived from these progenitors in vitro. The HbF content of marrow erythroblasts as determined by the same radioligand immunoassay was similar to that found in the peripheral blood, suggesting that the switch off of gamma-chain production occurs after the erythroid colony-forming unit stage of maturation. Increasing concentrations of a crude erythropoietin-containing preparation induced higher numbers of erythroid colonies, which were larger in size, but the HbF program was unaffected. In contrast to the hemoglobin accumulation in human progenitor-derived colonies, simian progenitor-derived colonies produced considerably more HbF, and the amount of HbF was strongly influenced by progenitor maturity. Assays of the HbF content of erythroblasts derived from culture of the peripheral blood BFU-E of patients with nondeletion hemoglobinopathies and their parents showed that the HbF program in the progenitors of such patients is highly variable. Some produce only a slight excess of HbF in progenitor-derived erythroblasts, whereas others have extraordinarily high HbF programs. The molecular basis of this variability is presently unknown.

Authors

A D Friedman, D C Linch, B Miller, J M Lipton, J Javid, D G Nathan

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Year: 2015 2001 1993 1990 1989 1987 1986 Total
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Citations to this article (10)

Title and authors Publication Year
Human primary erythroid cells as a more sensitive alternative in vitro hematological model for nanotoxicity studies: Toxicological effects of silver nanoparticles
N Rujanapun, S Aueviriyavit, S Boonrangsiman, A Rosena, D Phummiratch, S Riolueang, N Chalaow, V Viprakasit, R Maniratanachote 		Toxicology in Vitro 2015
Fetal and maternal progenitor cells in co-culture respond equally to erythropoietin
RM Bohmer, KL Johnson, DW Bianchi 		Prenatal Diagnosis 2001
A Short-Term Trial of Butyrate to Stimulate Fetal-Globin-Gene Expression in the β-Globin Disorders
SP Perrine, GD Ginder, DV Faller, GH Dover, T Ikuta, HE Witkowska, S Cai, EP Vichinsky, NF Olivieri 		New England Journal of Medicine 1993
Interferon-γ Modulates Fetal Hemoglobin Synthesis in Sickle Cell Anemia and Thalassemia
BA Miller, N Olivieri, SM Hope, DV Faller, SP Perrine 		Journal of Interferon Research 1990
Pharmacologic Manipulation of Fetal Hemoglobin in the Hemoglobinopathies
DG Nathan 		Annals of the New York Academy of Sciences 1990
Saudi Arabian Sickle Cell Anemia
BA Miller, M Salameh, M Ahmed, N Olivieri, TH Huisman, SH Orkin, DG Nathan 		Annals of the New York Academy of Sciences 1989
Application of high-performance liquid chromatographic methodology to the analysis of hemoglobins synthesized in erythroid progenitor cells
K Bhaumik, TH Huisman 		Journal of Chromatography B: Biomedical Sciences and Applications 1989
Human Ontogenic Development: Studies on the Hemopoietic System and the Expression of Homeo Box Genes
C Peschle 		Annals of the New York Academy of Sciences 1987
Butryic acid analogues augment γ globin gene expression in neonatal erythroid progenitors
SP Perrine, BA Miller, MF Greene, RA Cohen, N Cook, C Shackleton, DV Faller 		Biochemical and Biophysical Research Communications 1987
Investigations of the simian ontogenic switch from fetal to adult hemoglobin at the progenitor cell level
BP Alter, RS Weinberg, DC Linch, JM Schofield, BT Jackson, GJ Piasecki, JC Thornton, DG Nathan 		Journal of Clinical Investigation 1986

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