Preferential Binding of β^C Relative to β^S Globin to Stroma in Hemoglobin SC Disease

Abstract

Globin synthesis was measured in blood samples of four patients with hemoglobin SC Disease. In the stroma-free supernatant, globin synthesis was balanced with α/(β^S + β^C) = 1. In the exhaustively washed stroma, there was a preferential binding of β chains to stroma, similar to that reported for Sickle Cell Anemia. However, while the β^S/β^C ratio was balanced in the supernatant (0.8–0.9), the β^S/β^C ratio of the stroma varied from 0.2 to 0.4. Therefore, β^C is bound to stroma in preference to β^S in hemoglobin SC disease. These findings are consistent with the concept that binding of globin to stroma is related to the electrophoretic charge of the hemoglobin.