title: Interaction of alpha-thalassemia genes with each other and with HbC in an American black family.
abstract: 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.
mesh_terms: D000740:Anemia / Q000235:genetics; D044383:Black People; D001853:Bone Marrow / Q000378:metabolism; D004912:Erythrocytes / Q000378:metabolism; D005260:Female; D005914:Globins / Q000096:biosynthesis*; D006444:Hemoglobin C / Q000235:genetics*; D006454:Hemoglobins / Q000096:biosynthesis; D006801:Humans; D010375:Pedigree; D013789:Thalassemia / Q000235:genetics*
chemical_list: D006454:Hemoglobins; D005914:Globins; D006444:Hemoglobin C