The biosynthesis of hemoglobin G san josé (β 7 (A4) Glu→Gly)

This report is concerned with the evaluation of hematological parameters and the percentage level of the abnormal hemoglobin (Hb) G San José as found in 4 heterozygous carriers from a family of Sicilian origin. Biosynthetic studies and in vitro recombination experiments strongly indicate that abnormal β chains are synthesized at lower rate than βA chains and exhibit a minor affinity (relative to βA chains) for complementary chains in a condition of relative αA chain deficiency. The possibility that the low affinity of βG chains for α chains may play a decisive role in controlling the level of the abnormal Hb in the peripheral blood of the present non-α-thalassemic abnormal Hb carriers is therefore discussed. © 1981 S. Karger AG, Basel.