HB-PUNE - A NEW GAMMA-BETA-CROSSOVER HEMOGLOBIN FROM AN INDIAN FAMILY

We report studies on four members of an Indian family in which two chi ldren had clinical findings of transfusion dependent hemolytic anemia resembling thalassemia major but with fetal hemoglobin levels less tha n 2%. Hemoglobin A2 also was low. These parameters prompted us to furt her search for the type of hemoglobin. Cellulose acetate electrophores is at various pH and isoelectic levels focusing showed the presence of hemoglobin with similar electrophoretic mobility as hemoglobin A. Fin gerprinting of tryptic digests showed that gamma TP VII did not have h istidine reaction and that peptide therefore lacked histidine at posit ion 63. Fingerprinting showed the appearance of a new spot at a slight ly acidic position near beta TPIX and gamma TPV. Eluting this fragment showed residues of gamma TPV without residues of beta TPV and had asp artic acid, serine residues, and lysine. Peptides of this residues res embled beta TPIX peptides. Thus we have a chain showing gamma TPI to g amma TPVII followed by beta peptides i.e., beta TP VIII onwards. There thus has occurred a gamma beta crosover at gamma TPVII to beta TP VII I peptides.