Hemoglobin British Columbia [α₂β₂ 101(G3)Glu→Lys] a New Variant with High Oxygen Affinity

Abstract

Hemoglobin British Columbia was found in an East Indian living in Vancouver, British Columbia. Canada. Its structure was demonstrated to be α₂β₂ 101(G3)Glu→Lys. It has a significant increase in oxygen affinity, decrease in heme-heme interaction, but normal Bohr effect. Unlike Hb Rush (β101 Glu→Glu), it is as stable as Hb A to heat and alcohol denaturation. By both cellulose acetate electrophoresis and chromatography the undissociated Hb British Colmbia moves between Hb S and Hb A rather than behaving like Hb C. However, the dissociated abnormal β chain behaves like β^C. The substitution is at the α₂β₂ contact region. Except for a mild erythrocytosis, the propositus has normal hematological findings.