Henoglobin S-O ARAB-α-Thalassenia Globin Biosynmesis and Clinical Picture

Abstract
A 22 yr old American negro with mild anemia was triply heterozygous for Hb S, Hb O Arab and .alpha.-thalassemia. Hb A was not detected in the subject''s hemolysate. The .alpha.-thalassemia gene was expressed by an .alpha./non-.alpha. synthesis ratio of 0.71 .+-. 0.07 and was equally unbalanced in the peripheral blood and the bone marrow. The total radioactivity of the abnormal chains was equal, indicating equal rates of transcription and translation of the abnormal structural genes. Since the rate of synthesis of .beta.S and .beta.O was equal to the proportions of these abnormal chains in the peripheral blood, the abnormal gene products were equally stable. The mildness of the anemia, compared to that of the other reported S-O Arab doulbe heterozygotes, may be due to the moderating influence of the .alpha.-thal gene.