Sodium butyrate enhances fetal globin gene expression in erythroid progenitors of patients with Hb SS and β thalassemia

S. P. Perrine, Barbara Miller, D. V. Faller, R. A. Cohen, E. P. Vichinsky, D. Hurst, B. H. Lubin, T. Papayannopoulou

Research output: Contribution to journalArticle

104 Citations (Scopus)

Abstract

Increasing the expression of the γ globin genes is considered a useful therapeutic approach to the β globin diseases. Because butyrate and α-amino-n-butyric acid (ABA) augment γ globin expression in normal neonatal and adult erythroid progenitors, we investigated the effects of sodium butyrate and ABA on erythroid progenitors of patients with β thalassemia and sickle cell anemia who might benefit from such an effect. Both substances increased fetal hemoglobin (Hb F) expression in Bfu-e from 7% to 30% above levels found in control cultures from the same subjects with sickle cell anemia. The fraction of cultured erythroblasts producing Hb F increased more than 20% with sodium butyrate treatment in 70% of cultures. In most cultures, this produced > 20% total Hb F and > 70% F cells, levels which have been considered beneficial in ameliorating clinical symptoms. Alpha: non-alpha (α-non-α) imbalance was decreased by 36% in erythroid progenitors of patients with β thalassemia cultured in the presence of butyrate compared with control cultures from the same subjects. These data suggest that sodium butyrate may have therapeutic potential for increasing γ globulin expression in the β globin diseases.

Original languageEnglish (US)
Pages (from-to)454-459
Number of pages6
JournalBlood
Volume74
Issue number1
StatePublished - Jan 1 1989

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Thalassemia
Butyric Acid
Globins
Gene expression
Gene Expression
Fetal Hemoglobin
Butyrates
Sickle Cell Anemia
Erythroblasts
Globulins
Therapeutics
Genes

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Perrine, S. P., Miller, B., Faller, D. V., Cohen, R. A., Vichinsky, E. P., Hurst, D., ... Papayannopoulou, T. (1989). Sodium butyrate enhances fetal globin gene expression in erythroid progenitors of patients with Hb SS and β thalassemia. Blood, 74(1), 454-459.
Perrine, S. P. ; Miller, Barbara ; Faller, D. V. ; Cohen, R. A. ; Vichinsky, E. P. ; Hurst, D. ; Lubin, B. H. ; Papayannopoulou, T. / Sodium butyrate enhances fetal globin gene expression in erythroid progenitors of patients with Hb SS and β thalassemia. In: Blood. 1989 ; Vol. 74, No. 1. pp. 454-459.
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Perrine, SP, Miller, B, Faller, DV, Cohen, RA, Vichinsky, EP, Hurst, D, Lubin, BH & Papayannopoulou, T 1989, 'Sodium butyrate enhances fetal globin gene expression in erythroid progenitors of patients with Hb SS and β thalassemia', Blood, vol. 74, no. 1, pp. 454-459.

Sodium butyrate enhances fetal globin gene expression in erythroid progenitors of patients with Hb SS and β thalassemia. / Perrine, S. P.; Miller, Barbara; Faller, D. V.; Cohen, R. A.; Vichinsky, E. P.; Hurst, D.; Lubin, B. H.; Papayannopoulou, T.

In: Blood, Vol. 74, No. 1, 01.01.1989, p. 454-459.

Research output: Contribution to journalArticle

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Perrine SP, Miller B, Faller DV, Cohen RA, Vichinsky EP, Hurst D et al. Sodium butyrate enhances fetal globin gene expression in erythroid progenitors of patients with Hb SS and β thalassemia. Blood. 1989 Jan 1;74(1):454-459.