A New Hemoglobin with High Oxygen Affinity-Hemoglobin Bunbury: α₂β₂ [94 (Fg1) Asp→ Asn]

Abstract

Hemoglobin Bunbury was detected in an Italian woman from Bunbury, Western Australia, following detection of an abnormal hemoglobin by electrophoresis. The variant form accounted for 38% of the total hemoglobin and migrated on cellulose acetate electrophoresis between HbS and HbA. Structural analysis demonstrated a new substitution in the β chain, β94 Asp→ Asn. Hemoglobin Bunbury is stable to both heat and isopropanol denaturation. This variant shows an increase in oxygen affinity and a reduced Bohr effect. Heterozygosity for Hb Bunbury produces no clinical symptoms or compensatory erythrocytes is.