Unbalanced globin chain synthesis in erythroid precursor cells of heterozygous alpha-thalassaemia

Br J Haematol. 1976 Sep;34(1):55-60. doi: 10.1111/j.1365-2141.1976.tb00173.x.

Abstract

Globin biosynthesis was studied in both erythroid precursors and reticulocytes of three individuals with heterozygous alpha-thalassaemia. In contrast to the finding of equal or nearly equal alpha and beta chain synthesis in the marrow of patients with heterozygous beta-thalassaemia previously examined, our studies showed equal degrees of unbalanced globin synthesis in both reticulocytes and nucleated-erythroid cells of alpha-thalassaemia heterozygotes. Greater stability and less susceptibility to proteolysis of the excess beta-chain formed in alpha-thalassaemia may explain our findings.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Anemia, Sickle Cell / metabolism
  • Bone Marrow / metabolism*
  • Bone Marrow Cells*
  • Globins / biosynthesis*
  • Heterozygote
  • Humans
  • Iron / metabolism
  • Reticulocytes / metabolism
  • Thalassemia / genetics
  • Thalassemia / metabolism*

Substances

  • Globins
  • Iron