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

Abstract

In patients with heterozygous beta-thalassemia, the beta/alpha 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. alpha- and beta-chain synthesis is also equal in marrow cells in vivo. Equal synthesis is probably due both to a decrease in alpha-chain synthesis and an increase in beta-chain synthesis in bone marrow erythroid cells and may contribute to the absence of overt hemolysis due to excess alpha-globin chain accumulation in heterozygous beta-thalassemia.

• Authors: Y W Kan, D G Nathan, H F Lodish
• Journal: The Journal of clinical investigation (J Clin Invest) Vol. 51 Issue 7 Pg. 1906-9 (Jul 1972) ISSN: 0021-9738 [Print] United States
• PMID: 5032531 (Publication Type: Journal Article)

Chemical References

• Carbon Isotopes
• Globins
• Leucine

Topics

• Bone Marrow (metabolism)
• Bone Marrow Cells
• Carbon Isotopes
• Centrifugation, Density Gradient
• Globins (biosynthesis)
• Heterozygote
• Humans
• Leucine (metabolism)
• Peptide Biosynthesis
• Peptide Chain Elongation, Translational
• Reticulocytes (cytology, metabolism)
• Ribosomes (metabolism)
• Thalassemia (genetics, metabolism)