Skip to main content
PMC home page
The Journal of Clinical Investigation logoLink to The Journal of Clinical Investigation
. 1972 Jul;51(7):1906–1909. doi: 10.1172/JCI106993

Equal synthesis of α- and β-globin chains in erythroid precursors in heterozygous β-thalassemia

Yuet Wai Kan 1,2,3, David G Nathan 1,2,3, Harvey F Lodish 1,2,3
PMCID: PMC292339  PMID: 5032531

Abstract

In patients with heterozygous β-thalassemia, the β/α synthetic ratio in marrow erythroid cells incubated in vitro is 1, whereas in reticulocytes the ratio is 0.5. These ratios reflect the equal synthesis of the two chains on the polyribosomes of the bone marrow and unequal synthesis on the polyribosomes of the peripheral blood reticulocytes. α- and β-chain synthesis is also equal in marrow cells in vivo. Equal synthesis is probably due both to a decrease in α-chain synthesis and an increase in β-chain synthesis in bone marrow erythroid cells and may contribute to the absence of overt hemolysis due to excess α-globin chain accumulation in heterozygous β-thalassemia.

Full text

PDF
1906

Selected References

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

  1. Bank A., O'Donnell J. V. Intracellular loss of free alpha chains in beta thalassemia. Nature. 1969 Apr 19;222(5190):295–296. doi: 10.1038/222295a0. [DOI] [PubMed] [Google Scholar]
  2. Bargellesi A., Pontremoli S., Menini C., Conconi F. Excess of alpha-globin synthesis in homozygous beta-thalassemia and its removal from the red blood cell cytoplasm. Eur J Biochem. 1968 Jan;3(3):364–368. doi: 10.1111/j.1432-1033.1968.tb19538.x. [DOI] [PubMed] [Google Scholar]
  3. 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]
  4. Conconi F., Bargellesi A., Pontremoli S., Vigi V., Volpato S., Gaburro D. Absence of beta-globin synthesis and excess of gamma-globin synthesis in homozygous beta-thalassaemic subjects from the Ferrara region. Nature. 1968 Jan 20;217(5125):259–260. doi: 10.1038/217259a0. [DOI] [PubMed] [Google Scholar]
  5. Fessas P., Loukopoulos D., Kaltsoya A. Peptide analysis of the inclusions of erythroid cells in beta-thalassemia. Biochim Biophys Acta. 1966 Aug 24;124(2):430–432. doi: 10.1016/0304-4165(66)90216-9. [DOI] [PubMed] [Google Scholar]
  6. GAVOSTO F., MARAINI G., PILERI A. Radioautographic investigations on DNA and protein metabolism in 2 cases of Di Guglielmo's disease. Blood. 1960 Aug;16:1122–1132. [PubMed] [Google Scholar]
  7. HEYWOOD J. D., KARON M., WEISSMAN S. AMINO ACIDS: INCORPORATION INTO ALPHA- AND BETA-CHAINS OF HEMOGLOBIN BY NORMAL AND THALASSEMIC RETICULOCYTES. Science. 1964 Oct 23;146(3643):530–531. doi: 10.1126/science.146.3643.530. [DOI] [PubMed] [Google Scholar]
  8. Kan Y. W., Nathan D. G. Mild thalassemia: the result of interactions of alpha and beta thalassemia genes. J Clin Invest. 1970 Apr;49(4):635–642. doi: 10.1172/JCI106274. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Modell C. B., Latter A., Steadman J. H., Huehns E. R. Haemoglobin synthesis in beta-thalassaemia. Br J Haematol. 1969 Nov;17(5):485–501. doi: 10.1111/j.1365-2141.1969.tb01397.x. [DOI] [PubMed] [Google Scholar]
  10. NATHAN D. G., PIOMELLI S., GARDNER F. H. The synthesis of heme and globin in the maturing human erythroid cell. J Clin Invest. 1961 Jun;40:940–946. doi: 10.1172/JCI104333. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Nathan D. G., Gunn R. B. Thalassemia: the consequences of unbalanced hemoglobin synthesis. Am J Med. 1966 Nov;41(5):815–830. doi: 10.1016/0002-9343(66)90039-8. [DOI] [PubMed] [Google Scholar]
  12. Nathan D. G., Lodish H. F., Kan Y. W., Housman D. Beta thalassemia and translation of globin messenger RNA. Proc Natl Acad Sci U S A. 1971 Oct;68(10):2514–2518. doi: 10.1073/pnas.68.10.2514. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. 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]
  14. 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]

Articles from Journal of Clinical Investigation are provided here courtesy of American Society for Clinical Investigation

RESOURCES