Initiation of Globin Synthesis in β-Thalassemia

Ronald Crystal, Norton A. Elson, Arthur Nienhuis, Abigail C. Thornton, W. French Anderson

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Abstract

The mechanism of initiation of human globin chains was examined to determine if the initiation of protein synthesis is abnormal in β-thalassemia. Messenger RNA (mRNA) was isolated from the reticulocytes of normal adults, newborn infants and patients with β-thalassemia to study synthesis of alpha, beta and gamma chains. These isolated mRNA preparations were assayed in a cell-free system derived from rabbit reticulocytes. The initial dipeptides formed during the synthesis of the alpha and beta chains were determined. In normal subjects and patients with β-thalassemia the dipeptide for both the alpha and beta chains was found to be methionine-valine. The initial dipeptide for the gamma chain, determined in newborn infants and in patients with β-thalassemia, was methionine-glycine in both. All mRNA's studied shared a common requirement for methionyl-tRNAF (the specific initiator tRNA) and for the protein initiation factors. By these criteria, the mechanism of globin-chain initiation in β-thalassemia is normal.

Original languageEnglish
Pages (from-to)1091-1096
Number of pages6
JournalNew England Journal of Medicine
Volume288
Issue number21
DOIs
Publication statusPublished - 24 May 1973
Externally publishedYes

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ASJC Scopus subject areas

  • Medicine(all)

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Crystal, R., Elson, N. A., Nienhuis, A., Thornton, A. C., & Anderson, W. F. (1973). Initiation of Globin Synthesis in β-Thalassemia. New England Journal of Medicine, 288(21), 1091-1096. https://doi.org/10.1056/NEJM197305242882103