Absence of functional messenger RNA activity for beta globin chain
synthesis in beta 0-thalassemia
EJ Benz , PS Swerdlow and BG Forget
Department of Medicine, Children's Hospital Medical Center, Boston, Mass.
02115.
Functional human globin messenger RNA was isolated from reticulocytes of
two patients with homozygous beta 0-thalassemia, three patients with sickle
cell beta 0-thalassemia, and one patient doubly heterozygous for beta
0-thalassemia and hemoglobin Lepore. When incubated in the Krebs type II
mouse ascites tumor-cell-free system, messenger RNA from these patients
actively directed the synthesis of human beta s and/or alpha- and
gamma-globin chains but failed to stimulate the synthesis of any beta
A-chains, even though nonthalassemic human globin mRNA preparations
consistently stimulated two to four times as much beta A- or beta S-globin
chain synthesis as alpha-chain synthesis when incubated in the same system
under the same conditions. These results strongly suggest that functional
beta A-chain-specific globin mRNA is absent in beta 0-thalassemia.
Volume 45,
Issue 1,
pp. 1-10,
01/01/1975
Copyright © 1975 by The American Society of Hematology