Evidence for posttranslational control of Hb C synthesis in an individual
with Hb C trait and alpha-thalassemia
SA Liebhaber, FE Cash and DB Cornfield
Howard Hughes Medical Institute, University of Pennsylvania School of
Medicine, Philadelphia, PA 19104-6072.
The level of Hb C in the erythrocytes of individuals with Hb C trait
decreases significantly in the presence of coexisting alpha- thalassemia.
This relationship may result from the higher affinity of beta A than beta C
for limiting amounts of alpha-globin during hemoglobin assembly. This
mechanism would predict that the beta A and beta C synthetic capacity in
alpha-thalassemic individuals with Hb C trait should be balanced despite
the low levels of Hb C in their circulating erythrocytes. To directly test
this prediction, we have measured the beta A and beta C synthetic capacity
of reticulocyte RNA isolated from two individuals with Hb C trait, one with
a normal alpha- globin genotype and one with alpha-thalassemia. The
balanced expression of beta A and beta C in both cases supports the
proposed posttranslational control over Hb C expression.
Volume 71,
Issue 2,
pp. 502-504,
02/01/1988
Copyright © 1988 by The American Society of Hematology
