Interaction of α-thalassemia genes with each other and with HbC in an American black family

✍ Scribed by Brian K. Walker; Jean Atwater; Samir K. Ballas

• Book ID: 104788869
• Publisher: Springer
• Year: 1979
• Tongue: English
• Weight: 409 KB
• Volume: 17
• Category: Article
• ISSN: 0006-2928
• DOI: 10.1007/bf00504343

✦ Synopsis

The relative rates of in vitro synthesis of hemoglobin chains have been studied in an American black family in which the mother is doubly heterozygous for alpha-thalassemia and HbC and the father is heterozygous for alpha-thalassemia. The alpha/non-alpha synthetic ratio was equally unbalanced in both the bone marrow and the peripheral blood of the mother. Although HbC comprised 35% of her hemoglobin (compared to 42.2 +/- 2.2 in individuals with HbC trait and balanced globin synthesis), synthetic data showed that the newly synthesized beta C chain was 44% of the total newly synthesized beta chains. Isolated membranes contained more newly synthesized beta C than beta A chains. Three of the offspring were within the normal range, and the remaining three had alpha-thalassemia. There were two spontaneous abortions during the second trimester of pregnancy. Hydrops fetalis did not occur, and none of the children had HbH disease or HbC trait.