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The mechanism of autoxidation of oxyhemoglobin Kempsey (β99 asp → asn) was studied at pH 7.0, 37°C, using isoelectric focusing electrophoresis. During autoxidation, two intermediate hemoglobins, (α 2+ β 3+ ) 2 and (α 3+ β 2+ ) 2 were observed, and these were consecutively changed to...
We have studied patients with β-thalassemia from a Lebanese population having a high rate of consanguineous matings. This population exhibits both elevated and normal levels of Hb A2 associated with high levels of Hb F in homozygotes. The clinical course of the anemia suggests that this...
The use of isoelectric focusing in agarose gel was examined to test its usefulness for screening of cord blood hemoglobins and for the identification of hemoglobin variants in general. Advantages of agarose include: short running times, ease of preparation, and nontoxicity. in cord blood samples...
Hb Contaldo with a HIS→ARG substitution at position 103(G10) of the α chain is a newly discovered unstable Hb variant observed in an Italian child. Its instability is probably due to the disruption of the hydrogen bond between α103(G10)His and β108(G10)Asn. The structural variation in the...
A highly specific method for the conclusive identification of normal and variant human hemoglobins is described in this communication. The method employs the standard electrophoretic technique in combination with the immunoblot technique using monospecific antisera raised in rabbits. Various...
A new HbA 2 variant, HbA 2 Victoria δ24 (B6) Gly→Asp; was identified in a patient with β-thalassemia. Family studies indicated that the mutant δ gene was trans to the gene for β-thalassemia.
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