Publication Cover
Hemoglobin
international journal for hemoglobin research
Volume 31, 2007 - Issue 2
58
Views
3
CrossRef citations to date
0
Altmetric
Second Titus H.J. Huisman Memorial Symposium: Recent Advances in Hemoglobinopathy, May 8–9, 2006, Adana, Turkey

A Review of Cis-Trans Interplay Between DNA Sequences 5′ to the Gγ- and β-Globin Genes Among Hb F-Malta-I Heterozygotes/Homozygotes and β-Thalassemia Homozygotes/Compound Heterozygotes, and the Effects of Hydroxyurea on the Hb F/F-Erythrocyte; the Need for Large Multicenter Trials

, , , , , , , , , & show all
Pages 279-288 | Published online: 07 Jul 2009

REFERENCES

  • Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 1995; 332(20)1317–1322
  • Hankins JS, Ware RE, Rogers ZR, Wynn LW, Lane PA, Scott JP, Wang WC. Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood 2005; 106(7)2269–2275
  • Adragna NC, Fonseca P, Lauf PK. Hydroxyurea affects cell morphology, cation transport, and red blood cell adhesion in cultured vascular endothelial cells. Blood 1994; 83(2)553–560
  • Tyler JK. Chromatin assembly Cooperation between histone chaperones and ATP-dependent nucleosome remodeling machines. Eur J Biochem 2002; 269(9)2268–2274
  • Fucharoen S, Siritanaratkul N, Winichagoon P, Chowthaworn J, Siriboon W, Muangsup W, Chaicharoen S, Poolsup P, Chindavijak B, Pootrakul P, Piankijagum A, Rodgers GP. Hydroxyurea increases Hemoglobin F levels and improves the effectiveness of erythropoiesis in β-thalassemia/Hemoglobin E disease. Blood 1996; 87(3)887–892
  • Grosveld F, van Assendelft GB, Greaves DR, Kollias G. Position independent, high-level expression of the human β-globin gene in transgenic mice. Cell 1987; 51(6)975–985
  • Patrinos GP, de Krom M, de Boer E, Langeveld A, Imam AM, Strouboulis J, de Laat W, Grosveld FG. Multiple interactions between regulatory regions are required to stabilize an active chromatin hub. Genes Develop 2004; 18(12)1495–1509
  • Weatherall DJ, Clegg JB. The Thalassaemia Syndromes, 4th. Oxford, Blackwell Scientific Publications 2001
  • Huisman THJ, Jonxis JHP. The Hemoglobinopathies Techniques of Identification. Clinical and Biochemical Analysis. Marcel Dekker, Inc., New York 1977; 6
  • Bank A. Regulation of human fetal hemoglobin: new players, new complexities. Blood 2006; 107(2)435–443
  • Huisman THJ, Schroeder WA, Efremov GD, Duma H, Mladenovski B, Hyman CB, Rachmilewitz EA, Bouver N, Miller A, Brodie A, Shelton JB, Apell G. The present status of the heterogeneity of fetal hemoglobin in β-thalassemia: an attempt to unify some observations in thalassemia and related conditions. Ann NY Acad Sci 1973; 232: 107–124
  • Cauchi MN, Clegg JB, Weatherall DJ. Hemoglobin F(Malta): a new fetal hemoglobin variant with a high incidence in Maltese infants. Nature 1969; 223(203)311–313
  • Scerri CA, Abela W, Galdies R, Pizzuto ML, Grech JL, Felice AE. The β+IVS, I-NT no. 6 (T→C) thalassaemia in heterozygotes with an associated Hb Valletta or Hb S heterozygosity in homozygotes from Malta. Br J Haematol 1993; 83(4)669–671
  • Pulis S, Scerri C, Schembri Wismayer P, Galdies R, Bezzina Wettinger S, Felice AE. Developmental effect of the XmnI site on Gγ-globin gene expression among newborn Hb F-Malta-I [Gγ117(G19)His→Arg, CAT→CGT] heterozygotes and adult β+-thalassemia homozygotes. Hemoglobin 2007; 31(1)71–82
  • Miller SA, Dykes DD, Polesky HF. A simple salting out procedure for extracting DNA from human nucleated cells. Nucleic Acid Res 1988; 16(3)1215
  • Orkin SH, Kazazian HH, Jr, Antonarakis SE, Ostrer H, Goff SC, Sexton JP. Abnormal RNA processing due to the exon mutation of βE-globin gene. Nature 1982; 300(5894)768–769
  • Kutlar F, Felice AE, Grech JL, Bannister WH, Kutlar A, Wilson JB, Webber BB, Hu H, Huisman THJ. The linkage of Hb Valletta [α2β287(F3)Thr→Pro] and Hb F-Malta-I [α2Gγ2117(G19)His→Arg] in the Maltese population. Hum Genet 1991; 86(6)591–594
  • Berg PE, Mittelman M, Elion J, Labie D, Schechter AN. Increased protein binding to a −530 mutation of the human β-globin gene associated with decreased β-globin synthesis. Am J Hematol 1991; 36(1)42–47
  • Drew LR, Tang DC, Berg PE, Rodgers GP. The role of trans-acting factors and DNA-bending in the silencing of human β-globin gene expression. Nucleic Acids Res 2000; 28(14)2823–2830
  • Chase MB, Fu S, Haga SB, Davenport G, Stevenson H, Do K, Morgan D, Mah AL, Berg PE. BP1, a homeodomain-containing isoform of DLX4, represses the β-globin gene. Mol Cell Biol 2002; 22(8)2505–2514
  • Marwan MM, Scerri CA, Zarroag SO, Cao A, Kalogirou E, Kleanthous M, Ioannou P, Angastiniotis M, Felice AE. Comparative in vivo expression of β+-thalassemia alleles. Hemoglobin 1999; 23(3)221–229
  • DeSimone J, Heller P, Hall L, Wiers DZ. 5-Azacytidine stimulates fetal hemoglobin synthesis in anemic baboons. Proc Natl Acad Sci USA 1982; 79(14)4428–4431
  • Ley TJ, DeSimone J, Anagnou NP, Keller GH, Humphries RK, Turner PH, Young NS, Heller P, Nienhuis AW. 5-Azacytidine selectively increases γ-globin synthesis on a patient with β+-thalassemia. N Engl J Med 1982; 307(24)1469–1475
  • Mankidy R, Faller DV, Malbaaera R, Lowrey CH, Boosalis MS, White GL, Castaneda SA, Perrine SP. Short-chain fatty acids induce γ-globin gene expression bydisplacement of a HDAC3-NCoR repressor complex. Blood 2006; 108(9)3179–3186
  • Perrine SP, Greene MF, Faller DV. Delay in the fetal globin switch in infants of diabetic mothers. N Engl J Med 1985; 312(6)334–338
  • Torrealba-de Ron AT, Papayannopoulou Th, Knapp MS, Fu MF, Knitter G, Stamatoyannopoulos G. Perturbations in the erythroid marrow progenitor cell pools may play a role in the augmentation of HbF by 5-azacytidine. Blood 1984; 63(1)201–210
  • Saxon BR, Rees D, Olivieri NF. Regression of extramedullary haemopoiesis and augmentation of fetal haemoglobin concentration during hydroxyurea therapy in β thalassaemia. Br J Haematol 1998; 101(3)416–419
  • Galea M. Low dose hydroxyurea therapy in thalassaemia patients for the pharmacological induction of haemoglobin. B.Pharm Thesis, Department of Pharmacy, Faculty of Medicine and Surgery, University of Malta, MsidaMalta, 2005

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.