Hemoglobin synthesis in β-thalassemia: the properties of the free α-chains

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Abstract

The decrease in hemoglobin A (HbA, α2β2) synthesis in the erythroid cells of patients with β-thalassemia is due to a selective defect in β-chain synthesis. Since α-chains continue to be formed at a normal rate in these cells, this results in a marked relative excess of α-chain synthesis over β- and γ-chain synthesis. The α-chains uncombined with β- or β-like-chains (δ, γ) will be referred to as free α-chains. The experiments presented in this paper show that these free α-chains are capable of combining with β-chains to form HbA and are, therefore, structurally normal. Alternatively, in the absence of added β-chains, α-chains aggregates of various sizes are formed.

Authors

Arthur Bank