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Hemoglobin Athens‐Georgia [α 2 β 2 40(C6)Arg→Lys] in association with β 0 ‐thalassemia in Tunisia
Author(s) -
Mrad A.,
Kister J.,
Feo C.,
Poyart C.,
Kastally R.,
Blibech R.,
Galacteros F.,
Wajcman H.
Publication year - 1989
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830320208
Subject(s) - thalassemia , hemoglobin , hemoglobinopathy , oxygen , compound heterozygosity , heme , chemistry , biochemistry , medicine , hemolytic anemia , phenotype , enzyme , gene , organic chemistry
We describe an Hb Athens‐Georgia (Hb A‐Ga)/β 0 ‐thalassemia compound heterozygosity, found in a Tunisian patient. Oxygen binding studies of red cell suspensions of this patient, containing approximately 95% Hb A‐Ga, revealed an almost normal oxygen affinity. Nevertheless, dilute solutions of Hb A‐Ga showed an increased overall oxygen affinity and decreased heme‐heme interaction. This could be explained by a tetrameric hemoglobin with normal oxygen binding properties but with increased dissociation into monomers or dimers, as a consequence of a structural abnormality within the α 1 β 2 interface. Such an interpretation would explain the increased oxygen affinity reported in previous studies performed on heterozygous Hb A/Hb A‐Ga patients.