SEARCH

SEARCH BY CITATION

REFERENCES

  • Adunyah SE, Chander R, Barner VK, Cooper RS, Copper RS. 1995. Regulation of c-jun mRNA expression by hydroxyurea in human K562 cells during erythroid differentiation. Biochem Biophys Acta 1263: 123132.
  • Aguilar C, Vichinsky E, Neumayr L. 2005. Bone and joint disease in sickle cell disease. Hematol Oncol Clin North Am 19: 929941.
  • Al-Khatti A, Veith RW, Papayannopoulou T, Fritsch EF, Goldwasser E, Stamatoyannopoulos G. 1987. Stimulation of fetal hemoglobin synthesis by erythropoietin in baboons. N Engl J Med 317: 415420.
  • Asano H, Li XS, Stamatoyannopoulos G. 1999. FKLF, a novel Krü ppel-like factor that activates human embryonic and fetal β -like globin genes. Mol Cell Biol 19: 35713579.
  • Asano H, Li XS, Stamatoyannopoulos G. 2000. FKLF-2: a novel Krü ppel like transcriptional factor that activates globin and other erythroid lineage genes. Blood 95: 35783584.
  • Atweh GF, Sutton M, Nassif I, Boosalis V, Dover GJ, Wallenstein S, Wright E, McMahon L, Stamatoyannopoulos G, Faller DV, Perrine SP. 1999. Sustained induction of fetal hemoglobin by pulse butyrate therapy in sickle cell disease. Blood 93: 17901797.
  • Aufiero B, Neufeld EJ, Orkin SH. 1994. Sequence-specific DNA binding of individual cut repeats of the human CCAAT displacement/cut homeodomain protein. Proc Natl Acad Sci U S A 91: 77577761.
  • Bard H, Prosmanne J. 1985. Relative rates of fetal hemoglobin and adult hemoglobin synthesis in cord blood of infants of insulin-dependent diabetic mothers. Pediatrics 75: 11431147.
  • Bard H, Peri KG, Gagnon C. 2001. Changes in the G gamma- and A gamma-globin mRNA components of fetal hemoglobin during human development. Biol Neonate 80: 2629.
  • Behringer RR, Ryan TM, Palmiter RD, Brinster RL, Townes TM. 1990. Human γ - to β -globin gene switching in transgenic mice. Genes Dev 4: 380389.
  • Bhanu NV, Trice TA, Lee YT, Gantt NM, Oneal P, Schwartz JD, Noel P, Miller JL. 2005. A sustained and pancellular reversal of gamma-globin gene silencing in adult human erythroid precursor cells. Blood 105: 387393.
  • Bohmer RM. 2003. Reactivation of fetal hemoglobin in adult stem cell erythropoiesis by transforming growth factor-beta. J Hematother Stem Cell Res 12: 499504.
  • Bookchin RM, Nagel RL, Balaza T. 1975. Role of hybrid tetramer formation in gelation of haemoglobin S. Nature 256: 667668.
  • Bookchin RM, Balazs T, Nagel RL, Tellez I. 1977. Polymerisation of haemoglobin SA hybrid tetramers. Nature 269: 526527.
  • Boosalis MS, Ikuta T, Pace BS, da Fonseca S, White GL, Faller DV, Perrine SP. 1997. Abrogation of IL-3 requirements and stimulation of hematopoietic cell proliferation in vitro and in vivo by carboxylic acids. Blood Cells Mol Dis 23: 434442.
  • Boosalis MS, Bandyopadhyay R, Bresnick EH, Pace BS, Van DeMark K, Zhang B, Faller DV, Perrine SP. 2001. Short-chain fatty acid derivatives stimulate cell proliferation and induce STAT-5 activation. Blood 97: 32593267.
  • Browne P, Shalev O, Hebbel RP. 1998. The molecular pathobiology of cell membrane iron: the sickle red cell as a model. Free Radic Biol Med 24: 10401048.
  • Candido EP, Reeves R, Davie JR. 1978. Sodium butyrate inhibits histone deacetylation in cultured cells. Cell 14: 105113.
  • Cao H, Stamatoyannopoulos G, Jung M. 2004. Induction of human gamma globin gene expression by histone deacetylase inhibitors. Blood 103: 701709.
  • Carr BI, Reilly JG, Smith SS, Winberg C, Riggs A. 1984. The tumorigenicity of 5-azacytidine in the male Fischer rat. Carcinogenesis 5: 15831590.
  • Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, Vera JC, Levy PS. 1994. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood 84: 643649.
  • Chang YP, Maier-Redelsperger M, Smith KD, Contu L, Ducroco R, de Montalembert M, Belloy M, Elion J, Dover GJ, Girot R. 1997. The relative importance of the X-linked FCP locus and beta-globin haplotypes in determining haemoglobin F levels: a study of SS patients homozygous for beta S haplotypes. Br J Haematol 96: 806814.
  • Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR. 1995. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 332: 13171322.
  • Cheung JY, Miller BA. 2001. Molecular mechanisms of erythropoietin signaling. Nephron 87: 215222.
  • Cokic VP, Smith RD, Beleslin-Cokic BB, Njoroge JM, Miller JL, Gladwin MT, Schechter AN. 2003. Hydroxyurea induces fetal hemoglobin by the nitric oxide-dependent activation of soluble guanylyl cyclase. J Clin Invest 111: 231239.
  • Collins AF, Dover GJ, Luban NL. 1994. Increased fetal hemoglobin production in patients receiving valproic acid for epilepsy. Blood 84: 16901691.
  • Collins AF, Pearson HA, Giardina P, McDonagh KT, Brusilow SW, Dover GJ. 1995. Oral sodium phenylbutyrate therapy in homozygous beta thalassemia: a clinical trial. Blood 85: 4349.
  • Constantoulakis P, Papayannopoulou T, Stamatoyannopoulos G. 1988. alpha-Amino-N-butyric acid stimulates fetal hemoglobin in the adult. Blood 72: 19611967.
  • Craig JE, Rochette J, Fisher CA, Weatherall DJ, Marc S, Lathrop GM, Demenais F, Thein S. 1996. Dissecting the loci controlling fetal haemoglobin production on chromosomes 11p and 6q by the regressive approach. Nat Genet 12: 5864.
  • Crossley M, Whitelaw E, Perkins A, Williams G, Fujiwara Y, Orkin SH. 1996. Isolation and characterization of the cDNA encoding BKLF/TEF-2, a major CACCC-box-binding protein in erythroid cells and selected other cells. Mol Cell Biol 16: 16951705.
  • Darnell JE Jr. 1997. STATs and gene regulation. Science 277: 16301635.
  • DeSimone J, Heller P, Hall L, Zwiers D. 1982. 5-Azacytidine stimulates fetal hemoglobin synthesis in anemic baboons. Proc Natl Acad Sci U S A79: 44284431.
  • Dover GJ, Brusilow S, Charache S. 1994. Induction of fetal hemoglobin production in subjects with sickle cell anemia by oral sodium phenylbutyrate. Blood 84: 339343.
  • Downes SM, Hambleton IR, Chuang EL, Lois N, Serjeant GR, Bird AC. 2005. Incidence and natural history of proliferative sickle cell retinopathy observations from a cohort study. Ophthalmology 112: 18691875.
  • Ellis J, Tan-Un KC, Harper A, Michalovich D, Yannoutsos N, Philipsen S, Grosveld F. 1996. A dominant chromatin-opening activity in 5′ hypersensitive site 3 of the human beta-globin locus control region. EMBO J 15: 562568.
  • Enver T, Raich N, Ebens AJ, Papayannopoulou T, Costantini F, Stamatoyannopoulos G. 1990. Developmental regulation of human fetal to-adult globin gene switching in transgenic mice. Nature 344: 309313.
  • Ferry A, Baliga S, Monterio C, Chen Y, Pace BS. 1997. γ-Globin gene silencing in primary erythroid cultures: an inhibitory role for interleukin-6. J Biol Chem 272: 2003020037.
  • Fischer KD, Haese A, Nowock J. 1993. Cooperation of GATA-1 and Sp1 can result in synergistic transcriptional activation or interference. J Biol Chem 268: 2391523923.
  • Foley H, Ofori-Acquah S, Baliga BS, Pace BS. 2002. STAT3 mediates globin repression by Interleukin-6 in K562 cells. J Biol Chem 77: 1621116219.
  • Foulkes NS, Laoide BM, Schlotter F, Sassone-Corsi P. 1991. Transcriptional antagonist cAMP-responsive element modulator (CREM) down-regulates c-fos cAMP-induced expression. Proc Natl Acad Sci U S A 88: 54485452.
  • Forrester WC, Takegawa S, Papayannopoulou T, Stamatoyannopoulos G, Groudine M. 1987. Evidence for a locus activation region: the formation of developmentally stable hypersensitive sites in globin expressing hybrids. Nucleic Acids Res 15: 1015910177.
  • Fraser P, Grosveld F. 1998. Locus control regions, chromatin activation and transcription. Curr Opin Cell Biol 10: 361365.
  • Fucharoen S, Shimizu K, Fukumaki Y. 1990. A novel C-T transition within the distal CCAAT motif of the G gamma-globin gene in the Japanese HPFH: implication of factor binding in elevated fetal globin expression. Nucleic Acids Res 18: 52455253.
  • Gabbianelli M, Testa U, Massa A, Pelosi E, Sposi NM, Riccioni R, Luchetti L, Peschle C. 2000. 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 95: 35553561.
  • Galanello R, Stamatoyannopoulos G, Papayannopoulou T. 1988. Mechanism of Hb F stimulation by S-stage compounds. In vitro studies with bone marrow cells exposed to 5-azacytidine, Ara-C, or hydroxyurea. J Clin Invest 81: 12091216.
  • Garner C, Silver N, Best S, Menzel S, Martin C, Spector TD, Thein SL. 2004. Quantitative trait locus on chromosome 8q influences the switch from fetal to adult hemoglobin. Blood 104: 21842186.
  • Gommerman JL, Sittaro D, Klebasz NZ, Williams DA, Berger SA. 2000. Differential stimulation of c-Kit mutants by membrane-bound and soluble Steel Factor correlates with leukemic potential. Blood 96: 37343742.
  • Goodwin AJ, McInerney JM, Glander MA, Pomerantz O, Lowrey CH. 2001. In vivo formation of a human beta-globin locus control region core element requires binding sites for multiple factors including GATA-1, NF-E2, erythroid Kruppel-like factor, and Sp1. J Biol Chem 276: 2688326892.
  • Grosveld F, van Assendelft GB, Greaves DR, Kollias G. 1987. Position independent, high-level expression of the human β -globin gene in transgenic mice. Cell 51: 975985.
  • Gubin AN, Njoroge JM, Bouffard GG, Miller JL. 1999. Gene expression in proliferating human erythroid cells. Genomics 59: 168177.
  • Gumucio DL, Rood KL, Gray TA, Riordan MF, Sartor CI, Collins FS. 1988. Nuclear proteins that bind the human gamma-globin gene promoter: alterations in binding produced by point mutations associated with hereditary persistence of fetal hemoglobin. Mol Cell Biol 8: 53105322.
  • Gustin JA, Pincheira R, Mayo LD, Ozes ON, Kessler KM, Baerwald MR, Korgaonkar CK, Donner DB. 2004. Tumor necrosis factor activates CRE-binding protein through a p38 MAPK/MSK1 signaling pathway in endothelial cells. Am J Physiol Cell Physiol 286: C547C555.
  • Haby C, Lisovoski F, Aunis D, Zwiller J. 1994. Stimulation of the cyclic GMP pathway by NO induces expression of the immediate early genes c-fos and junB in PC12 cells. J Neurochem 62: 496501.
  • Hankins JS, Ware RE, Rogers ZR, et al., 2005. Long-term hydroxyurea therapy for infants with sickle cell anemia - the HUSOFT extension study. Blood 106: 22692275.
  • Hardison R, Riemer C, Chui DH, Huisman TH, Miller W. 1998. Electronic access to sequence alignments, experimental results, and human mutations as an aid to studying globin gene regulation. Genomics 47: 429437.
  • Haynes J Jr, Baliga BS, Obiako B, Ofori-Acquah S, Pace B. 2004. Zileuton induces hemoglobin F synthesis in erythroid progenitors: role of the L-arginine-nitric oxide signaling pathway. Blood 103: 39453950.
  • Hazzalin CA, Cano E, Cuenda A, Barratt MJ, Cohen P, Mahadevan LC. 1996. p38/RK is essential for stress-induced nuclear responses: JNK/SAPKs and c-Jun/ATF-2 phosphorylation are insufficient. Curr Biol 6: 10281031.
  • Hodges D, Coghill E, Keys J, Mauire T, Hartmann B, McDowall A, Weiss M, Grimmond S, Perkins A. 2005. A global role for EKLF in definitive and primitive erythropoiesis. Blood [Epub ahead of print].
  • Ikuta T, Kan YW, Swerdlow PS, Faller DV, Perrine SP. 1998. Alterations in protein-DNA interactions in the gamma-globin gene promoter in response to butyrate therapy. Blood 92: 29242933.
  • Ikuta T, Ausenda S, Cappellini MD. 2001. Mechanism for fetal globin gene expression: role of the soluble guanylate cyclase-cGMP-dependent protein kinase pathway. Proc Natl Acad Sci U S A 98: 18471852.
  • Ingram VM. 1957. Gene mutations in human haemoglobin: the chemical difference between normal and sickle haemoglobin. Nature 180: 326328.
  • Jackson DA, McDowell JC, Dean A. 2003. Beta-globin locus control region HS2 and HS3 interact structurally and functionally. Nucleic Acids Res 31: 11801190.
  • Jane SM, Gumucio DL, Ney PA, Cunningham JM, Nienhuis AW. 1993. Methylation-enhanced binding of Sp1 to the stage selector element of the human gamma-globin gene promoter may regulate development specificity of expression. Mol Cell Biol 13: 32723281.
  • Jane SM, Nienhuis AW, Cunningham JM. 1995. Hemoglobin switching in man and chicken is mediated by a heteromeric complex between the ubiquitous transcription factor CP2 and a developmentally specific protein. EMBO J 14: 97105.
  • Johnson J, Hunter R, McElveen R, Qian XH, Baliga BS, Pace BS. 2005. Fetal hemoglobin induction by the histone deacetylase inhibitor, scriptaid. Cell Mol Biol 51: 229238.
  • Kan YW, Dozy AM. 1980. Evolution of the hemoglobin S and C genes in world populations. Science 209: 388391.
  • Kim-Shapiro DB, King SB, Bonifant CL, Kolibash CP, Ballas SK. 1998. Time resolved absorption study of the reaction of hydroxyurea with sickle cell hemoglobin. Biochim Biophys Acta 1380: 6474.
  • King BS. 2003. Arole for nitric oxide in hydroxyurea-mediated fetal hemoglobin induction. J Clin Invest 111: 171172.
  • Kollias G, Wrighton N, Hurst J, Grosveld F. 1986. Regulated expression of human Aγ -, β -, and hybrid β γ - globin genes in transgenic mice: manipulation of the developmental expression patterns. Cell 46: 8994.
  • Koshy M, Dorn L, Bressler L, Molokie R, Lavelle D, Talischy N, Hoffman R, van Overveld W, DeSimone J. 2000. 2-deoxy 5-azacytidine and fetal hemoglobin induction in sickle cell anemia. Blood 96: 23792384.
  • Kulozik AE, Kar BC, Satapathy RK, Serjeant BE, Serjeant GR, Weatherall DJ. 1987. Fetal hemoglobin levels and beta (s) globin haplotypes in an Indian populations with sickle cell disease. Blood 69: 17421741742176.
  • Kutlar A, Hattori Y, Bakioglu I, Kutlar F, Kamel K, Huisman TH. 1985. Hematological observations on Arabian SS patients with a homozygosity or heterozygosity for a beta S chromosome with haplotype #31. Hemoglobin 9: 545557.
  • Krauss JS, Freant LJ, Lee JR. 1998. Gastrointestinal pathology in sickle cell disease. Ann Clin Lab Sci 28: 1923.
  • Kruh J. 1982. Effects of sodium butyrate, a new pharmacological agent, on cells in culture. Mol Cell Biochem 42: 6582.
  • Kuo MH, Allis CD. 1998. Roles of histone acetyltransferases and deacetylases in gene regulation. Bioessays 20: 615626.
  • Lapoumeroulie C, Dunda O, Ducrocq R, Trabuchet G, Mony-Lobe M, Bodo JM, Carnevale P, Labie D, Elion J, Krishnamoorthy R. 1992. A novel sickle cell mutation of yet another origin in Africa: the Cameroon type. Hum Genet 89: 333337.
  • Lavelle DE. 2004. The molecular mechanism of fetal hemoglobin reactivation. Semin Hematol 41: 310.
  • Lee YT, Miller LD, Gubin AN, Makhlouf F, Wojda U, Barrett AJ, Liu ET, Miller JL. 2001. Transcription patterning of uncoupled proliferation and differentiation in myelodysplastic bone marrow with erythroid-focused arrays. Blood 98: 19141921.
  • Letvin NL, Linch DC, Beardsley GP, McIntyre KW, Nathan DG. 1984. Augmentation of fetal-hemoglobin production in anemic monkeys by hydroxyurea. N Engl J Med 310: 869873.
  • Ley TJ, DeSimone J, Noguchi CT, Turner PH, Schechter AN, Heller P, Nienhuis AW. 1983. 5-Azacytidine increases gamma-globin synthesis and reduces the proportion of dense cells in patients with sickle cell anemia. Blood 62: 370380.
  • Liakopoulou E, Blau CA, Li Q, Josephson B, Wolf JA, Fournarakis B, Raisys V, Dover G, Papayannopoulou T, Stamatoyannopoulos G. 1995. Stimulation of fetal hemoglobin production by short chain fatty acids. Blood 86: 32273235.
  • Liu DP, Liang CC, Ao ZH, Jia PC, Chen SS, Wang RX, Liu LJ, Jin HQ, Zha DY, Huang YW. 1990. Treatment of severe beta-thalassemia (patients) with myleran. Am J Hematol 33: 5055.
  • Liu LR, Du ZW, Zhao HL, Liu XL, Huang XD, Shen J, Ju LM, Fang FD, Zhang JW. 2005. T to C substitution at −175 or −173 of the gamma-globin promoter affects GATA-1 and OCT-1 binding in vitro differently but can independently reproduce the hereditary persistance of fetal hemoglobin phenotype intransgenic mice. J Biol Chem 280: 74527459.
  • Maier-Redelsperger M, Noguchi de CT, Montalembert M, Rodgers GP, Schechter AN, Gourbil A, Blanchard D, Jais JP, Ducrocq R, Peltier JY. 1994. Variation in fetal hemoglobin parameters and predicted hemoglobin S polymerization in sickle cell children in the first two years of life: Parisian Prospective Study on Sickle Cell Disease. Blood 84: 31823188.
  • Mantovani R, Malgaretti N, Nicolis S, Ronchi A, Giglioni B, Ottolenghi S. 1988. The effects of HPFH mutations in the human gamma-globin promoter on binding of ubiquitous and erythroid specific nuclear factors. Nucleic Acids Res 16: 77837797.
  • Marotta CA, Forget BG, Cohen-Solal M, Weissman SM. 1976. Nucleotide sequence analysis of coding and noncoding regions of human beta-globin mRNA. Prog Nucleic Acid Res Mol Biol 19: 165175.
  • Martin DI, Orkin SH. 1990. Transcriptional activation and DNA binding by the erythroid factor GF-1/NF-E1/Eryf 1. Genes Dev 4: 18861898.
  • Mata F, Rius C, Cabanas C, Bernabeu C, Aller P. 1989. S-phase inhibitors induce vimentin expression in human promonocytic U-937 cells. FEBS Lett 259: 171174.
  • Mavilio F, Giampaolo A, Care A, Migliaccio G, Calandrini M, Russo G, Pagliardi GL, Mastroberardino G, Marinucci M, Peschle C. 1983. Molecular mechanisms of human hemoglobin switching: selective undermethylation and expression of globin genes in embryonic, fetal, and adult erythroblasts. Proc Natl Acad Sci U S A 80: 69076911.
  • McCaffrey PG, Newsome DA, Fibach E, Yoshida M, Su MS. 1997. Induction of gamma-globin by histone deacetylase inhibitors. Blood 90: 20752083.
  • McDonagh KT, Lin HJ, Lowrey CH, Bodine DM, Nienhuis AW. 1991. The upstream region of the human gamma-globin gene promoter. Identification and functional analysis of nuclear protein binding sites. J Biol Chem 266: 1196511974.
  • McElveen RL, Lou TF, Reese K, Xia S, Baliga BS, Pace BS. 2005. Erk pathway inhibitor U0126 induces gamma-globin expression in erythroid cells. Cell Mol Biol 51: 215227.
  • Miller IJ, Bieker JJ. 1993. A novel, erythroid cell-specific murine transcription factor that binds to the CACCC element and is related to the Kruppel family of nuclear proteins. Mol Cell Biol 13: 27762786.
  • Miller BA, Salameh M, Ahmed M, Wainscoat J, Antognetti G, Orkin S, Weatherall D, Nathan DG. 1986. High fetal hemoglobin production in sickle cell anemia in the eastern province of Saudi Arabia is genetically determined. Blood 67: 14041410.
  • Mizzen CA, Allis CD. 1998. Linking histone acetylation to transcriptional regulation. Cell Mol Life Sci 54: 620.
  • Munugalavadla V, Kapur R. 2005. Role of c-Kit and erythropoietin receptor in erythropoiesis. Crit Rev Oncol Hematol 54: 6375.
  • Nagel RL, Bookchin RM, Johnson J, Labie D, Wajcman H, Isaac-Sodeye WA, Honig GR, Schiliro G, Crookston JH, Matsutomo K. 1979. Structural bases of the inhibitory effects of Hb F and A2 on the polymerization of Hb S. Proc Natl Acad Sci U S A 76: 670672.
  • Nagel RL, Fabry ME, Pagnier J Pagnier J, Zohoun I, Wajcman H, Baudin V, Labie D. 1985. Hematologically and genetically distinct forms of sickle cell anemia in Africa. The Senegal type and the Benin type. N Engl J Med 312: 880884.
  • Nagel RL, Rao SK, Dunda-Belkhodja O, Connolly MM, Fabry ME, Georges A, Krishnamoorthy R, Labie D. 1987. The hematologic characteristics of sickle cell anemia bearing the Bantu haplotype: the relationship between G gamma and HbF level. Blood 69: 10261030.
  • Nagel RL, Erlingsson S, Fabry ME, Fabry ME, Croizat H, Susuka SM, Lachman H, Sutton M, Driscoll C, Bouhassira E, Billett, HH. 1991. The Senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients. Blood 77: 13711375.
  • Nagel RL, Vichinsky E, Shah M, Johnson R, Spadacino E, Fabry ME, Mangahas L, Abel R, Stamatoyannopoulos G. 1993. F reticulocyte response in sickle cell anemia treated with recombinant human erythropoietin: a double-blind study. Blood 81: 914.
  • New L, Jiang Y, Zhao M, Liu K, Zhu W, Flood LJ, Kato Y, Parry GC, Han J. 1998. PRAK, a novel protein kinase regulated by the p38 MAP kinase. EMBO J 17: 33723384.
  • Ney PA, Sorrentino BP, Lowrey CH, Nienhuis AW. 1990. Inducibility of the HS II enhancer depends on binding of an erythroid specific nuclear protein. Nucleic Acids Res 18: 60116017.
  • Nolan VG, Wyszynski DF, Farrer LA, Steinberg MH. 2005. Hemolysis associated priapism in sickle cell disease. Blood 106: 32643267.
  • Norton VG, Imai BS, Yau P, Bradbury EM. 1989. Histone acetylation reduces nucleosome core particle linking number change. Cell 57: 449457.
  • Ofori-Acquah SF, Lalloz MRA, Serjeant G, Layton DM. 2004. Dominant influence of gamma-globin promoter polymorphisms on fetal haemoglobin expression in sickle cell disease. Cell Mol Biol 50: 3542.
  • Pace BS, Li Q, Stamatoyannopoulos G. 1996. In vivo search for butyrate responsive sequences using transgenic mice carrying A gamma gene promoter mutants. Blood 88: 10791083.
  • Pace BS, White GL, Dover GJ, Boosalis MS, Faller DV, Perrine SP. 2002. Short-chain fatty acid derivatives induce fetal globin expression and erythropoiesis in vivo. Blood 100: 46404648.
  • Pace BS, Qian XH, Sangerman J, Ofori-Acquah SF, Baliga BS, Han J, Critz SD. 2003. p38 MAP kinase activation mediates gamma-globin gene induction in erythroid progenitors. Exp Hematol 31: 10891096.
  • Papayannopoulou T, Torrealba de Ron A, Veith R, Knitter G, Stamatoyannopoulos G. 1984. Arabinosylcytosine induces fetal hemoglobin in baboons by perturbing erythroid cell differentiation kinetics. Science 224: 617619.
  • Papayannopoulou T, Nakamoto B, Kurachi S, Nelson R. 1987. Analysis of the erythroid phenotype of HEL cells: clonal variation and the effect of inducers. Blood 70: 17641772.
  • Park JI, Choi HS, Jeong JS, Han JY, Kim IH. 2001. Involvement of p38 kinase in hydroxyurea-induced differentiation of K562 cells. Cell Growth Differ 12: 481486.
  • Perrine SP, Rudolph A, Faller DV, Roman C, Cohen RA, Chen SJ, Kan YW. 1988. Butyrate infusions in the ovine fetus delay the biologic clock for globin gene switching. Proc Natl Acad Sci U S A 85: 85408542.
  • Perrine SP, Swerdlow P, Faller DV, Qin G, Rudolph AM, Reczek J, Kan YW. 1989. Butyric acid modulates developmental globin gene switching in man and sheep. Adv Exp Med Biol 271: 177183.
  • Perrine SP, Ginder GD, Faller DV, Dover GH, Ikuta T, Witkowska HE, Cai SP, Vichinsky EP, Olivieri NF. 1993. A short-term trial of butyrate to stimulate fetal-globin-gene expression in the beta-globin disorders. N Engl J Med 328: 8186.
  • Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, Kinney TR. 1991. Pain in sickle cell disease. Rates and risk factors. N Engl J Med 325: 1116.
  • Powars DR. 1990. Sickle cell anemia and major organ failure. Hemoglobin 14: 573598.
  • Prengler M, Pavlakis SG, Prohovnik I, Adams RJ. 2002. Sickle cell disease: the neurological complications. Ann Neurol 51: 543552.
  • Ristaldi MS, Drabek D, Gribnau J, Poddie D, Yannoutsous N, Cao A, Grosveld F, Imam AM. 2001. The role of the -50 region of the human gamma-globin gene in switching. EMBO J 20: 52425249.
  • Ronchi A, Nicolis S, Santoro C, Ottolenghi S. 1989. Increased Sp1 binding mediates erythroid-specific overexpression of a mutated (HPFH) gamma-globulin promoter. Nucleic Acids Res 17: 1023110241.
  • Sampietro M, Thein SL, Contreras M, Pazmany L. 1992. Variation of Hb F and F-cell number with the G-gamma Xmn I (C-T) polymorphism in normal individuals. Blood 79: 832833.
  • Saunthararajah Y, Hillery CA, Lavelle D, Molokie R, Dorn L, Bressler L, Gavazova S, Chen YH, Hoffman R, DeSimone J. 2003. Effects of 5-aza-2′-deoxycytidine on fetal hemoglobin levels, red cell adhesion, and hematopoietic differentiation in patients with sickle cell disease. Blood 102: 38653870.
  • Schaefer TS, Sanders LK, Nathans D. 1995. Cooperative transcriptional activity of Jun and Stat3 beta, a short form of Stat3. Proc Natl Acad Sci U S A 92: 90979101.
  • Schroeder WA, Huisman TH, Shelton JB, Kleihauer EF, Dozy AM, Roberson B. 1968. Evidence for multiple structural genes for the gamma chain of human fetal hemoglobin. Proc Natl Acad Sci U S A 60: 537544.
  • Skalnik DG, Strauss EC, Orkin SH. 1991. CCAAT displacement protein as a repressor of the myelomonocytic-specific gp91-phox gene promoter. J Biol Chem 266: 1673616744.
  • Stamatoyannopoulos G, Josephson B, Zhang JW, Li Q. 1993. Developmental regulation of human gamma-globin genes in transgenic mice. Mol Cell Biol 13: 76367644.
  • Stamatoyannopoulos G, Grosveld F. 2001. Hemoglobin switching. In: StamatoyannopoulosG, MajerusPW, PerlmutterRM, VarmusH, editors. The molecular basis of blood disease. Vol. 3. Philadelphia: Saunders.
  • Steinberg MH, Lu ZH, Barton FB, Terrin ML, Charache S, Dover GJ. 1997. Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Multicenter Study of Hydroxyurea. Blood 89: 10781088.
  • Stoming TA, Stoming GS, Lanclos KD, Fei YJ, Altay C, Kutlar F, Huisman TH. 1989. An A gamma type of nondeletional hereditary persistence of fetal hemoglobin with a T[RIGHTWARDS ARROW] C mutation at position -175 to the cap site of the A gamma globin gene. Blood 73: 329333.
  • Strouboulis J, Dillon N, Grosveld F. 1992. Developmental regulation of a complete 70-kb human β -globin locus in transgenic mice. Genes Dev 6: 18571864.
  • Suhasini M, Boss GR, Pascual FE, Pilz RB. 1995. Nitric oxide-releasing agents and cGMP analogues inhibit murine erythroleukemia cell differentiation and suppress erythroid-specific gene expression: correlation with decreased DNA binding of NF-E2 and altered c-myb mRNA expression. Cell Growth Differ 6: 15591566.
  • Syed RS, Reid SW, Li C, Cheetham JC, Aoki KH, Liu B, Zhan H, Osslund TD, Chirino AJ, Zhang J, Finer-Moore J, Elliott S, Sitney K, Katz BA, Matthews DJ, Wendoloski JJ, Egrie J, Stroud RM. 1998. Efficiency of signaling through cytokine receptors depends critically on receptor orientation. Nature 395: 511516.
  • Szafraniec SI, Stachnik KJ, Skierski JS. 2004. New nucleoside analogs in the treatment of solid tumors. Acta Pol Pharm 61: 297305.
  • Tanabe O, Katsuoka F, Campbell AD, Song W, Yamamoto M, Tanimoto K, Engel JD. 2002. An embryonic/fetal beta-type globin gene repressor contains a nuclear receptor TR2/TR4 heterodimer. EMBO J 21: 34343442.
  • Tang DC, Zhu J, Liu W, Chin K, Sun J, Chen L, Hanover JA, Rodgers GP. 2005. The hydroxyurea-induced small GTP-binding protein SAR modulates gamma-globin gene expression in human erythroid cells. Blood 106: 32563263.
  • Thommes K, Lennartsson J, Carlberg M, Ronnstrand L. 1999. Identification of Tyr-703 and Tyr-936 as the primary association sites for Grb2 and Grb7 in the c-Kit/stem cell factor receptor. Biochem J 341: 211216.
  • Topley JM, Rogers DW, Stevens MC, Serjeant GR. 1981. Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. Arch Dis Child 56: 765769.
  • Torkelson S, White B, Faller DV, Phipps K, Pantazis C, Perrine SP. 1996. Erythroid progenitor proliferation is stimulated by phenoxyacetic and phenylalkyl acids. Blood Cells Mol Dis 22: 150158.
  • Torrealba-de Ron AT, Papayannopoulou T, Knapp MS, Fu MF, Knitter G, Stamatoyannopoulos G. 1984. Perturbations in the erythroid marrow progenitor cell pools may play a role in the augmentation of HbF by 5-azacytidine. Blood 63: 201210.
  • Townes TM, Behringer RR. 1990. Human globin locus activation region (LAR): role in temporal control. Trends Genet 6: 219223.
  • Tsang AP, Visvader JE, Turner CA, Fujiwara Y, Yu C, Weiss MJ, Crossley M, Orkin SH. 1997. FOG, a multitype zinc finger protein, acts as a cofactor for transcription factor GATA-1 in erythroid and megakaryocytic differentiation. Cell 90: 109119.
  • Tsang AP, Fujiwara Y, Hom DB, Orkin SH. 1998. Failure of megakaryopoiesis and arrested erythropoiesis in mice lacking the GATA-1 transcriptional cofactor FOG. Genes Dev 12: 11761188.
  • Turner BM. 1991. Histone acetylation and control of gene expression. J Cell Sci 99: 1320.
  • Uddin S, Ah-Kang J, Ulaszek J, Mahmud D, Wickrema A. 2004. Differentiation stage-specific activation of p38 mitogen-activated protein kinase isoforms in primary human erythroid cells. Proc Natl Acad Sci U S A 101: 147152.
  • Ulrich MJ, Gray WJ, Ley TJ. 1992. An intramolecular DNA triplex is disrupted by point mutations associated with hereditary persistence of fetal hemoglobin. J Biol Chem 267: 1864918658.
  • Vaishnav D, Jambal P, Reusch JE, Pugazhenthi S. 2003. SP600125, an inhibitor of c-jun N-terminal kinase, activates CREB by a p38 MAPK-mediated pathway. Biochem Biophys Res Commun 307: 855960.
  • van der Ploeg LH, Flavell RA. 1980. DNA methylation in the human gamma delta beta-globin locus in erythroid and nonerythroid tissues. Cell 19: 947958.
  • Veith R, Papayannopoulou T, Kurachi S, Stamatoyannopoulos G. 1985. Treatment of baboon with vinblastine: insights into the mechanisms of pharmacologic stimulation of Hb F in the adult. Blood 66: 456459.
  • Wall L, Destroismaisons N, Delvoye N, Guy LG. 1996. CAAT/enhancer-binding proteins are involved in beta-globin gene expression and are differentially expressed in murine erythroleukemia and K562 cells. J Biol Chem 271: 1647716484.
  • Watowich SS, Wu H, Socolovsky M, Klingmuller U, Constantinescu SN, Lodish HF. 1996. Cytokine receptor signal transduction and the control of hematopoietic cell development. Annu Rev Cell Dev Biol 12: 91128.
  • Watson J, Stahman AW, Bilello FP. 1948. The significance of the paucity of sickle cells in newborn Negro infants. Am J Med Sci 215: 419423.
  • Weinberg RS, Ji X, Sutton M, Perrine S, Galperin Y, Li Q, Liebhaber SA, Stamatoyannopoulos G, Atweh GF. 2005. Butyrate increases the efficiency of translation of gamma-globin mRNA. Blood 105: 18071809.
  • Wenzel SE, Kamada AK. 1996. Zileuton: the first 5-lipoxygenase inhibitor for the treatment of asthma. Ann Pharmacother 30: 858864.
  • Witt O, Sand K, Pekrun A. 2000. Butyrate-induced erythroid differentiation of human K562 leukemia cells involves inhibition of ERK and activation of p38 MAP kinase pathways. Blood 95: 23912396.
  • Witt O, Monkemeyer S, Kanbach K, Pekrun A. 2002. Induction of fetal hemoglobin synthesis by valproate: modulation of MAP kinase pathways. Am J Hematol 71: 4547.
  • Witt O, Monkemeyer S, Ronndahl G, Erdlenbruch B, Reinhardt D, Kanbach K, Pekrun A. 2003. Induction of fetal hemoglobin expression by the histone deacetylase inhibitor apicidin. Blood 101: 20012007.
  • Wojchowski DM, Gregory RC, Miller CP, Pandit AK, Pircher TJ. 1999. Signal transduction in the erythropoietin receptor system. Exp Cell Res 253: 143156.
  • Wood WG. 1993. Increased HbF in adult life. Baillieres Clin Haematol 6: 177213.
  • Wood WG, Bunch C, Kelly S, Gunn Y, Breckon G. 1985. Control of haemoglobin switching by a developmental clock. Nature 313: 320323.
  • Zhao Q, Cumming H, Cerruti L, Cunningham JM, Jane SM. 2004. Site-specific acetylation of the fetal globin activator NF-E4 prevents its ubiquitination and regulates its interaction with the histone deacetylase, HDAC1. J Biol Chem 279: 4147741486.
  • Zimmerman SA, Schultz WH, Davis JS, Pickens CV, Mortier NA, Howard TA. 2004. Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood 103: 20392045.