Hemoglobin Athens-Georgia [alpha 2 beta 2 40(C6)Arg----Lys] in association with beta 0-thalassemia in Tunisia.
We describe an Hb Athens-Georgia (Hb A-Ga)/beta 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 alpha 1 beta 2 interface. Such an interpretation would explain the increased oxygen affinity reported in previous studies performed on heterozygous Hb A/Hb A-Ga patients.
Mrad
A
A
Laboratoire d'Hématologie, Hôpital Habib Thameur, Tunis, Tunisia.
Kister
J
J
Feo
C
C
Poyart
C
C
Kastally
R
R
Blibech
R
R
Galacteros
F
F
Wajcman
H
H
D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013789:Thalassemia / Q000097:blood*; D014416:Tunisia
D006455:Hemoglobins, Abnormal; C060131:hemoglobin Athens-Georgia