Hemoglobin British Columbia (alpha2beta2 101(G3)Glu replaced by Lys). A new variant with high oxygen affinity.

R T Jones, B Brimhall, G Gray

Hemoglobin 1976-1977

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Hemoglobin British Columbia was found in an East Indian living in Vancouver, British Columbia, Canada. Its structure was demonstrated to be alpha2beta2 101(G3)Glu replaced by Lys. It has a significant increase in oxygen affinity, decrease in heme-heme interaction, but normal Bohr effect. Unlike Hb Rush (beta101 Glu replaced by Glu), it is as stable as Hb A to heat and alcohol denaturation. By both cellulose acetate electrophoresis and chromatography the undissociated Hb British Columbia moves between Hb S and Hb A rather than behaving like Hb C. However, the dissociated abnormal beta chain behaves like beta C. The substitution is at the alpha2beta2 contact region. Except for a mild erythrocytosis, the propositus has normal hematological findings.

Citation

R T Jones, B Brimhall, G Gray. Hemoglobin British Columbia (alpha2beta2 101(G3)Glu replaced by Lys). A new variant with high oxygen affinity. Hemoglobin. 1976-1977;1(2):171-82