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. 1979 Aug;16(4):296–301. doi: 10.1136/jmg.16.4.296

Globin chain synthesis in sickle beta-thalassaemic bone marrow and reticulocytes.

S Ladas, G Chalevelakis, C Lyberatos, E Vaidakis, G Arapakis
PMCID: PMC1012673  PMID: 490583

Abstract

Globin chain synthesis was studied in reticulocytes and bone marrow erythroid precursors in four sickle beta-thalassaemic Greek patients. Significant globin chain imbalance was found in reticulocytes (alpha/gamma + beta A + beta S = 2.20 +/- SD 0.16) and bone marrow (alpha/gamma + beta A + beta S = 1.58 +/- SD 0.11) after two hours' incubation. There was evidence of contamination of the gamma, beta A, and, to a lesser extent, of the beta S chain by non-haem proteins. The contamination was more obvious in chromatograms obtained from whole cell bone marrow samples and could partially explain the lower alpha/non-alpha ratio found in bone marrow.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bank A., Braverman A. S., Marks P. A. Globin chain synthesis in thalassemia. Ann N Y Acad Sci. 1969 Nov 20;165(1):231–237. doi: 10.1111/j.1749-6632.1969.tb27793.x. [DOI] [PubMed] [Google Scholar]
  2. Bank A., Dow L. W., Farace M. G., O'Donnell J. V., Ford S., Natta C. Changes in globin synthesis with erythroid cell maturation in sickle thalassemia. Blood. 1973 Mar;41(3):353–357. [PubMed] [Google Scholar]
  3. Bank A., Mears G., Weiss R., O'Donnell J. V., Natta C. Preferential binding of beta s globin chains associated with stroma in sickle cell disorders. J Clin Invest. 1974 Oct;54(4):805–809. doi: 10.1172/JCI107820. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Braverman A. S., Bank A. Changing rates of globin chain synthesis during erythroid cell maturation in thalassemia. J Mol Biol. 1969 May 28;42(1):57–64. doi: 10.1016/0022-2836(69)90486-0. [DOI] [PubMed] [Google Scholar]
  5. Braverman A. S., Bank A. Hemoglobin synthesis during erythroid cell development in thalassemia and other hemolytic anemias. Ann N Y Acad Sci. 1969 Nov 20;165(1):295–304. doi: 10.1111/j.1749-6632.1969.tb27799.x. [DOI] [PubMed] [Google Scholar]
  6. Chalevelakis G., Clegg J. B., Weatherall D. J. Globin synthesis in normal human bone marrow. Br J Haematol. 1976 Dec;34(4):535–557. doi: 10.1111/j.1365-2141.1976.tb03600.x. [DOI] [PubMed] [Google Scholar]
  7. Chalevelakis G., Clegg J. B., Weatherall D. J. Imbalanced globin chain synthesis in heterozygous beta-thalassemic bone marrow. Proc Natl Acad Sci U S A. 1975 Oct;72(10):3853–3857. doi: 10.1073/pnas.72.10.3853. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Clegg J. B., Naughton M. A., Weatherball D. J. Abnormal human haemoglobins. Separation and characterization of the alpha and beta chains by chromatography, and the determination of two new variants, hb Chesapeak and hb J (Bangkok). J Mol Biol. 1966 Aug;19(1):91–108. doi: 10.1016/s0022-2836(66)80052-9. [DOI] [PubMed] [Google Scholar]
  9. Clegg J. B., Weatherall D. J. Haemoglobin synthesis during erythroid maturation in -thalassaemia. Nat New Biol. 1972 Dec 6;240(101):190–192. doi: 10.1038/newbio240190a0. [DOI] [PubMed] [Google Scholar]
  10. DeSimone J., Adams J. G., 3rd, Shaeffer J. Evidence for rapid loss of newly synthesized haemoglobin S molecules in sickle cell anaemia and sickle cell trait. Br J Haematol. 1977 Mar;35(3):373–385. doi: 10.1111/j.1365-2141.1977.tb00597.x. [DOI] [PubMed] [Google Scholar]
  11. Friedman S., Oski F. A., Schwartz E. Bone marrow and peripheral blood globin synthesis in an American black family with beta thalassemia. Blood. 1972 Jun;39(6):785–793. [PubMed] [Google Scholar]
  12. Gill F. M., Schwartz E. Free alpha-globin pool in human bone marrow. J Clin Invest. 1973 Dec;52(12):3057–3063. doi: 10.1172/JCI107504. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Gill F. M., Schwartz E. Synthesis of globin chains in sickle -thalassemia. J Clin Invest. 1973 Mar;52(3):709–714. doi: 10.1172/JCI107232. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Heywood D., Karon M., Weissman S. Asymmetrical incorporation of amino acids in the alpha and beta chains of hemoglobin synthesized by thalassemic reticulocytes. J Lab Clin Med. 1965 Sep;66(3):476–482. [PubMed] [Google Scholar]
  15. Kan Y. W., Nathan D. G., Lodish H. F. Equal synthesis of - and -globin chains in erythroid precursors in heterozygous -thalassemia. J Clin Invest. 1972 Jul;51(7):1906–1909. doi: 10.1172/JCI106993. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Nienhuis A. W., Canfield P. H., Anderson W. F. Hemoglobin messenger RNA from human bone marrow. Isolation and translation in homozygous and heterozygous beta-thalassemia. J Clin Invest. 1973 Jul;52(7):1735–1745. doi: 10.1172/JCI107355. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Rieder R. F. Globin chain synthesis in HbD (Punjab)-beta-thalassemia. Blood. 1976 Jan;47(1):113–120. [PubMed] [Google Scholar]
  18. SCHROEDER W. A. THE HEMOGLOBINS. Annu Rev Biochem. 1963;32:301–320. doi: 10.1146/annurev.bi.32.070163.001505. [DOI] [PubMed] [Google Scholar]
  19. Schwartz E. Heterozygous Beta thalassemia: balanced globin synthesis in bone marrow cells. Science. 1970 Mar 13;167(3924):1513–1514. doi: 10.1126/science.167.3924.1513. [DOI] [PubMed] [Google Scholar]
  20. Shchory M., Ramot B. Globin chain synthesis in the marrow and reticulocytes of beta thalassemia, hemoglobin H disease, and beta delta thalassemia. Blood. 1972 Jul;40(1):105–111. [PubMed] [Google Scholar]
  21. Vettore L., De Matteis M. C., Bassetto M. A. Globin chain synthesis in human bone marrow. Br J Haematol. 1977 Jun;36(2):297–297. doi: 10.1111/j.1365-2141.1977.tb00650.x. [DOI] [PubMed] [Google Scholar]
  22. Weatherall D. J., Clegg J. B., Na-Nakorn S., Wasi P. The pattern of disordered haemoglobin synthesis in homozygous and heterozygous beta-thalassaemia. Br J Haematol. 1969 Mar;16(3):251–267. doi: 10.1111/j.1365-2141.1969.tb00400.x. [DOI] [PubMed] [Google Scholar]
  23. Weatherall D. J., Clegg J. B., Naughton M. A. Globin synthesis in thalassaemia: an in vitro study. Nature. 1965 Dec 11;208(5015):1061–1065. doi: 10.1038/2081061a0. [DOI] [PubMed] [Google Scholar]
  24. White J. M., Lang A., Lehmann H. Compensation of chain synthesis by the single chain gene in Hb Lepore trait. Nat New Biol. 1972 Dec 27;240(104):271–273. doi: 10.1038/newbio240271a0. [DOI] [PubMed] [Google Scholar]
  25. Wood W. G., Stamatoyannopoulos G. Globin synthesis in fractionated Normoblasts of beta-thalassemia heterozygotes. J Clin Invest. 1975 Mar;55(3):567–578. doi: 10.1172/JCI107964. [DOI] [PMC free article] [PubMed] [Google Scholar]

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