Pharmacologic induction of fetal hemoglobin synthesis: Cellular and molecular mechanisms

Pediatric Pathology and Molecular Medicine

Volumn 20, Issue 1, 2001, Pages 87-106

  Yang, Y M ^a   Pace, B ^a  

^a Department of Pediatrics ^*  (United States)

Author keywords

Fetal hemoglobin; Hemoglobinopathy; Induction; Pharmacologic; globin

Indexed keywords

5 AZA 2' DEOXYCYTIDINE; AZACITIDINE; BETA GLOBIN; BUSULFAN; BUTYRIC ACID DERIVATIVE; ERYTHROPOIETIN; GAMMA GLOBIN; HEMOGLOBIN A; HEMOGLOBIN ALPHA CHAIN; HEMOGLOBIN BETA CHAIN; HEMOGLOBIN F; HEMOGLOBIN S; HYDROXYUREA; GLOBIN; TRANSCRIPTION FACTOR;

CHROMATIN STRUCTURE; CHROMOSOME 11; CHROMOSOME 16; CLINICAL TRIAL; DRUG EFFICACY; DRUG MECHANISM; ERYTHROPOIESIS; GENE LOCUS; GLOBIN GENE; GLOBIN SYNTHESIS; HEMOGLOBIN SYNTHESIS; HUMAN; MOLECULAR BIOLOGY; PRIORITY JOURNAL; PROTEIN INDUCTION; REVIEW; SICKLE CELL ANEMIA; TRANSCRIPTION REGULATION; BIOSYNTHESIS; BLOOD; DRUG EFFECT; FORECASTING; GENE EXPRESSION REGULATION; GENETICS; PHYSIOLOGY; REGULATOR GENE;

ANEMIA, SICKLE CELL; AZACITIDINE; BUSULFAN; BUTYRATES; ERYTHROPOIESIS; ERYTHROPOIETIN; FETAL HEMOGLOBIN; FORECASTING; GENE EXPRESSION REGULATION; GENE EXPRESSION REGULATION, DEVELOPMENTAL; GENES, SWITCH; GLOBINS; HUMAN; HYDROXYUREA; SUPPORT, U.S. GOV'T, P.H.S.; TRANSCRIPTION FACTORS;

EID: 85047682960     PISSN: 15227952     EISSN: None     Source Type: Journal    
DOI: 10.1080/15513810109168819     Document Type: Review

References (132)

1. Hemoglobin switching
2. Cellular regulation of hemoglobin switching; evidence for inverse relationship between fetal hemoglobin synthesis and degree of maturity of human erythroid cells
3. Proportion of fetal hemoglobin synthesis decreases during erythroid cell maturation
4. Quantitation of hemoglobins within individual red cells: Asynchronous biosynthesis of fetal and adult hemoglobin during erythroid maturation in normal subjects
5. Erythropoiesis and hemoglobin production: A unifying model involving sequential gene activation
6. The asynchrony of γ- and β-chain synthesis during human erythroid cell maturation
7. Increased HbF in adult life
8. Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2
9. Gender and haplotype effects upon hematological manifestations of adult sickle cell anemia
10. Relationship of foetal haemoglobin levels and β haplotypes in homozygous sickle cell disease
11. An analysis of fetal hemoglobin variation in sickle cell disease: The relative contributions of X-linked factor, β-globin haplotypes, α-globin gene number, gender and age
12. The patterns of fetal haemoglobin production in leukaemia
13. Stimulation of fetal hemoglobin synthesis in baboons by hemolysis and hypoxia
14. Production of erythrocytes that contain fetal hemoglobin in anemia
15. Fetal erythropoiesis in stress hematopoiesis
16. Fetal Hb production during acute erythroid expansion. I. Observations in patients with transient erythroblastopenia and post-phlebotomy
17. Acceleration of F-cell production in response to experimentally induced anemia in adult baboons (Papio cynocephalus)
18. Genetic relationship between fetal Hb levels in normal and erythropoietically stressed baboons
19. Maintenance of fetal hemoglobin (Hb F) elevations in the baboon by prolonged erythropoietic stress
20. Hb F production in stressed erythropoiesis: Observations and kinetic models
21. Maternal synthesis of haemoglobin F in pregnancy
22. Production of erythrocytes that contain fetal hemoglobin in anemia
23. Studies of Hb F in adult nonanemic baboons: Hb F expression in erythroid colonies decreases as the level of maturation of erythroid progenitors advances
24. Studies on human foetal haemoglobin. II. Foetal haemoglobin levels in healthy children and adults and in certain haematological disorders
25. Evolution of the hemoglobin S and C genes in world population
26. High fetal hemoglobin production in sickle cell anemia in the eastern province of Saudi Arabia is genetically determined
27. A novel sickle gene of yet another origin in Africa: The Cameroon type
28. Hematological observations on Arabian SS patients with a homozygosity or heterozygosity for a beta S chromosome with haplotype #31
29. Fetal hemoglobin levels and beta S globin haplotypes in an Indian population with sickle cell disease
30. Gγ and HbF level
31. Gγ globin production
32. Sequence variations in the 5′ flanking and IVS II regions of the Gγ and Aγ globin genes of β S chromosomes with five different haplotypes
33. o-thalassemia: Three types observed in South-Chinese families
34. Gγ expression
35. Molecular analysis of the high-hemoglobin F phenotype in Saudi Arabian sickle cell anemia
36. Variation in hemoglobin F production among normal and sickle cell adults is not related to nucleotide substitutions in the γ promoter regions
37. Black β-thalassemia homozygotes with specific sequence variations in the 5′ hypersensitive site-2 of the locus control region have high levels of fetal hemoglobin
38. Gγ levels
39. Genetic control of F cells in human adults
40. The significance of the paucity of sickle cells in newborn Negro infants
41. o-thalassemia: A comparison with sickle cell anemia
42. oHPFH conditions in combination with β-thalassemia and HBS
43. Sickle cell anemia as a syndrome: A review of diagnostic features
44. Sickle cell anemia and trait in southern India: Further studies
45. Natural history of sickle cell anemia in Saudi Arabs: A study of 270 subjects
46. The spectrum of beta-thalassemia mutations on the Indian subcontinent: The basis for prenatal diagnosis
47. Heterogeneity and variation of clinical and haematological expression of haemoglobin S in Saudi Arabs
48. Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia?
49. Pain in sickle cell disease. Rates and risk factors
50. The painful crisis of homozygous sickle cell disease. A study of risk factors
51. Mortality in sickle cell disease
52. Fetal hemoglobin, sickling, and sickle cell disease
53. Mortality in children and adolescents with sickle cell disease
54. Nucleation-controlled aggregation of deoxyhemoglobin S. Participation of hemoglobin F in the aggregation of deoxyhemoglobin S in concentrated phosphate buffer
55. Nucleation-controlled aggregation of deoxyhemoglobin S. Possible difference in the size of nuclei in different phosphate concentrations
56. Hydroxyurea: Effects on hemoglobin F production in patients with sickle cell anemia
57. Hydroxyurea for the treatment of sickle cell disease
58. Treatment of sickle cell anemia with hydroxyurea and erythropoietin
59. Effects of hydroxyurea on hemoglobin F and water content in the red blood cells of dogs and of patients with sickle cell anemia
60. Hematologic responses of patients with sickle cell disease to treatment with hydroxyurea
61. Quantitative changes in globin mRNA levels during hydroxyurea treatment of sickle cell anemia and beta-thalassemia
62. Effects of hydroxyurea on the rheological properties of sickle erythrocytes in vivo
63. Treatment of sickle cell anemia with hydroxyurea and erythropoietin
64. Hydroxyurea affects cell morphology, cation transport, and red cell adhesion in cultured vascular endothelial cells
65. The effect of methemoglobin formation in sickle cell disease
66. Medroxyprogesterone acetate and homozygous sickle cell disease
67. 5-Azacytidine stimulates fetal hemoglobin synthesis in anemic baboons
68. Treatment of sickle cell anemia with 5-azacytidine results in increased fetal hemoglobin production and is associated with nonrandom hypomethylation of DNA around the gamma-delta-beta-globin gene complex
69. 5-Azacytidine increases gamma-globin synthesis and reduces the proportion of dense cells in patients with sickle cell anemia
70. 5-Azacytidine increases HbF production and reduces anemia in sickle cell disease: Dose-response analysis of subcutaneous and oral dosage regimens
71. Editorial: 5-azacytidine for beta-thalassemia?
72. 5-Azacytidine and 5-aza-2′-deoxycytidine behave as different antineoplastic agents in B16 melanoma
73. Augmentation of fetal hemoglobin (HbF) levels by low-dose short-duration 5′-AZA-2-deoxycytidine (decitabine) administration in sickle cell anemia patients who had no HbF elevation following hydroxyurea therapy
74. Augmentation of fetal hemoglobin production in anemic monkeys by hydroxyurea
75. Hydroxyurea effects on hemoglobin F production in patients with sickle cell anemia
76. Hematologic responses of patients with sickle cell disease to treatment with hydroxyurea
77. Stimulation of F-Cell production in patients with sickle cell anemia treated with cytarabine or hydroxyurea
78. Treatment of severe beta-thalassemia patients with Myleran
79. Treatment of baboon with vinblastine: Insights into the mechanisms of pharmacologic stimulation of HbF in the adult
80. Stimulation of fetal hemoglobin synthesis by erythropoietin in baboons
81. Hydroxyurea-induced HbF production in anemic primates: Augmentation by erythropoietin, hematopoietic growth factors and sodium butyrate
82. Augmentation by erythropoietin of the fetal-hemoglobin response to hydroxyurea in sickle cell disease
83. F reticulocyte response in sickle cell anemia treated with recombinant human erythropoietin: A double blind study
84. Effect of interleukin-3 and erythropoietin on in vivo erythropoiesis and F-cell formation in primates
85. Delay in the fetal globin switch in infants of diabetic mothers
86. On the indication of fetal hemoglobin by butyrates: In vivo and in vitro studies with sodium butyrate and comparison of combination treatments with 5 AzaC and AraC
87. A short-term trial of butyrate to stimulate fetal-globin-gene expression in the beta-globin disorders
88. Increased fetal hemoglobin in patients receiving sodium-4-phenybutyrate
89. Sodium phenybutyrate increases fetal hemoglobin production in patients with sickle cell disease
90. Cellular regulation of hemoglobin switching: Evidence for inverse relationship between fetal hemoglobin synthesis and degree of maturity of human erythroid cells
91. Relationship of burst-forming unit-erythroid progenitors and their DNA-synthesis stage to fetal hemoglobin levels in hydroxyurea-treated patients with sickle cell anemia
92. BFU-E colony growth in response to hydroxyurea: Correlation between in vitro and in vivo fetal hemoglobin induction
93. Hydroxyurea induction of hemoglobin F production in sickle cell disease: Relationship between cytotoxicity and F cell production
94. Hb F production in stressed erythropoiesis: Observations and kinetic models
95. Perturbations in the erythroid marrow progenitor cell pools may play a role in the augmentation of Hb F by 5-azacytidine
96. 5-Azacytidine acts directly on both erythroid precursors and progenitors to increase production of fetal hemoglobin
97. Mechanism of Hb F stimulatin by S-stage compounds: In vitro studies with bone marrow cells exposed to 5-azacytidine, ara-C or hydroxyurea
98. Influence of hydroxyurea on fetal hemoglobin production in vitro
99. Hemoglobin F synthesis in vitro: Evidence for control at the level of primitive erythroid stem cells
100. Fetal hemoglobin and the erythroid stem cell differentiation process
101. Stimulation of fetal hemoglobin synthesis by erythropoietin in baboons
102. Fetal hemoglobin synthesis in vivo: Direct evidence for control at the level of erythroid progenitors
103. Cell biology of hemoglobin switching I. The switch from fetal to adult hemoglobin formation during ontogeny
104. Hb F production in stressed erythropoiesis: Observations and kinetic models
105. Developmental regulation of human γ- and β-globin genes in the absence of the locus control region
106. The β-like-globin gene domain inhuman erythroid cells
107. Position-independent, high-level expression of the human β-globin gene in transgenic mice
108. Regulation of globin gene expression in erythroid cells
109. NP-E2 and GATA binding motifs are required for the formation of DNase I hypersensitivity site 4 of the human β-globin locus control region
110. Increased γ-globin expression in a nondeletion HPFH mediated by an erythroid specific DNA-binding factor
111. Erythroid transcription factor NF-E2 is a haematopoietic-specific basic leucine zipper protein
112. The erythroid Kruppel-like factor transactivation domain is a critical component for cell specific inducibility of a β-globin promoter
113. FKLF, a novel Kruppel-like factor that activates human embryonic and fetal β-like globin genes
114. Identification of a stage selector element in the human γ-globin gene promoter that fosters preferential interaction with 5′ HS2 enhance when in competition with the β-promoter
115. Heterogeneity in the molecular basis of hereditary persistence of fetal hemoglobin
116. +-HPFH
117. The essentials of DNA methylation
118. Fetal hemoglobin reactivation in baboon and man. A short perspective
119. DNA methylation in the human γδβ-globin locus in erythroid and nonerythroid cells
120. Methylation enhanced binding of Sp1 to the stage selector element of the human γ-globin gene promoter may regulate developmental specificity of expression
121. Locus control region-Aγ transgenic mice: A new model for studying the induction of fetal hemoglobin in the adult
122. Transgenic mouse model of pharmacologic induction of fetal hemoglobin: Studies using a new ribonucleotide reductase inhibitor, Didox
123. Overview of pathophysiology and rationale for treatment of sickle cell anemia
124. Sodium butyrate inhibits histone deacetylation in cultured cells
125. Induction of γ-globin by histone deacetylase inhibitors
126. Induction of the human γ-globin gene promoter in K562 cells by sodium butyrate: Reversal of repression by CCAAT displacement protein
127. Alterations in protein-DNA interactions in the γ-globin gene promoter in response to butyrate therapy
128. In vivo search for butyrate responsive sequences using transgenic mice carrying Aγ gene promoter mutants
129. Butyrate inducible elements in the human γ globin gene promoter
130. Sodium butyrate induces tyrosine phosphorylation and activation of MAP kinase (ERK-1) in human K562 cells
131. Abrogation of IL-3 requirements and stimulation of hematopoietic cell proliferation in vitro and in vivo by carboxylic acids
132. Drug discovery for hemoglobinopathies: High-throughput screening and assays for inducers of fetal hemoglobin