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RM Bookchin and T Balazs
Factors contributing to the clinical differences between sickle cell-
hemoglobin C disease (SC) and the benign sickle cell trait (AS) include the
higher proportion of hemoglobin (Hb) S and the higher cell Hb
concentrations in SC compared with AS red cells. Reports differ, however,
about whether Hb C copolymerizes more than Hb A with Hb S when measured by
minimum gelling concentrations (MGCs) and polymer solubilities of the
deoxy-Hb mixtures. We now show that the MGCs and solubilities of equimolar
mixtures of Hb S + Hb C vary much more with the ionic strength (mu) of the
solution than those of Hb S + Hb A mixtures. At mu less than or equal to
0.20, but not at mu greater than 0.25, Hb S + Hb C solubilities were
significantly lower than those of Hb S + Hb A. These differences which may
reflect a greater effect of the beta 6Lys+ in Hb C at lower mu, can account
for the reported discrepancies. The solubility differences were similar in
the presence or absence of asymmetric hybrids, and since the
intratetramerically cross-linked hybrids alpha 2 beta s beta A and alpha 2
beta s beta c had similar solubilities, they did not indicate the usual
mechanism, involving greater incorporation of alpha 2 beta s beta c into
the polymers. The small solubility differences between the two Hb mixtures
at physiologic (red cell) concentrations of Hb and 2,3- diphosphoglycerate
probably play a minor role in the clinical differences between SC and AS
states.
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| Copyright © 1986 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
