title: Hemoglobin British Columbia (alpha2beta2 101(G3)Glu replaced by 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 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.
mesh_terms: D000328:Adult; D001955:British Columbia; D002852:Chromatography, Ion Exchange; D004588:Electrophoresis, Cellulose Acetate; D014644:Genetic Variation; D005973:Glutamine / Q000097:blood; D006455:Hemoglobins, Abnormal / Q000302:isolation & purification*; D006801:Humans; D008239:Lysine / Q000097:blood; D008297:Male; D010108:Oxyhemoglobins
chemical_list: D006455:Hemoglobins, Abnormal; D010108:Oxyhemoglobins; D005973:Glutamine; D008239:Lysine