Hemoglobin switching in unicellular erythroid culture of sibling erythroid burst-forming units: kit ligand induces a dose-dependent fetal hemoglobin reactivation potentiated by sodium butyrate

Blood. 2000 Jun 1;95(11):3555-61.

Abstract

Mechanisms underlying fetal hemoglobin (HbF) reactivation in adult life have not been elucidated; particularly, the role of growth factors (GFs) is controversial. Interestingly, histone deacetylase (HD) inhibitors (sodium butyrate, NaB, trichostatin A, TSA) reactivate HbF. We developed a novel model system to investigate HbF reactivation: (1) single hematopoietic progenitor cells (HPCs) were seeded in serum-free unilineage erythroid culture; (2) the 4 daughter cells (erythroid burst-forming units, [BFU-Es]), endowed with equivalent proliferation/differentiation and HbF synthesis potential, were seeded in 4 unicellular erythroid cultures differentially treated with graded dosages of GFs and/or HD inhibitors; and (3) HbF levels were evaluated in terminal erythroblasts by assay of F cells and gamma-globin content (control levels, 2.4% and 1.8%, respectively, were close to physiologic values). HbF was moderately enhanced by interleukin-3 (IL-3) and granulocyte-macrophage colony-stimulating factor treatment (up to 5%-8% gamma-globin content), while sharply reactivated in a dose-dependent fashion by c-kit ligand (KL) and NaB (20%-23%). The stimulatory effects of KL on HbF production and erythroid cell proliferation were strictly correlated. A striking increase of HbF was induced by combined addition of KL and NaB or TSA (40%-43%). This positive interaction is seemingly mediated via different mechanisms: NaB and TSA may modify the chromatin structure of the beta-globin gene cluster; KL may activate the gamma-globin promoter via up-modulation of tal-1 and possibly FLKF transcription factors. These studies indicate that KL plays a key role in HbF reactivation in adult life. Furthermore, combined KL and NaB administration may be considered for sickle cell anemia and beta-thalassemia therapy.

MeSH terms

  • Adult
  • Butyrates / pharmacology*
  • Cell Culture Techniques / methods
  • Cell Differentiation / drug effects
  • Cells, Cultured
  • Erythrocytes / cytology
  • Erythrocytes / drug effects
  • Erythrocytes / physiology*
  • Erythropoietin / pharmacology
  • Fetal Hemoglobin / biosynthesis*
  • Genes, Switch*
  • Globins / biosynthesis
  • Granulocyte-Macrophage Colony-Stimulating Factor / pharmacology
  • Hematopoietic Stem Cells / cytology
  • Hematopoietic Stem Cells / drug effects
  • Hematopoietic Stem Cells / physiology*
  • Hemoglobin A / biosynthesis*
  • Humans
  • Interleukin-3 / pharmacology
  • Male
  • Recombinant Proteins / pharmacology
  • Stem Cell Factor / pharmacology*

Substances

  • Butyrates
  • Interleukin-3
  • Recombinant Proteins
  • Stem Cell Factor
  • Erythropoietin
  • Granulocyte-Macrophage Colony-Stimulating Factor
  • Globins
  • Hemoglobin A
  • Fetal Hemoglobin