Hemoglobin I in an American Negro family: Structural and hematologic studies

An American Negro family is described, in which the mother and two children have hemoglobin I trait, each with 24 to 28 per cent hemoglobin I. In all three of these subjects, target cells comprise about 10 per cent of the erythrocyte population. No other hematologic abnormality was found in the parents and their five examined children. In tests with 4 per cent sodium metabisulfite, changes resembling sickling occurred in the erythrocytes of all seven members of the family and also in five unrelated individuals who are homozygous for hemoglobin A. On structural analysis, the substitution in this sample of hemoglobin I is α¹⁶ lys → glu, as was previously found in one other sample of hemoglobin I (I Philadelphia).