FAILURE OF ALPHA-THALASSEMIA GENE TO DECREASE SEVERITY OF SICKLE-CELL ANEMIA
- 1 January 1978
- journal article
- research article
- Vol. 51 (6) , 1163-1168
Abstract
A 15 yr old black male with homozygous sickle cell disease was severely growth retarded amd had a chronic hemolytic anemia requiring transfusions. Globin chain synthetic studies of peripheral blood reticulocytes and bone marrow cells revealed a ratio of .alpha. to .beta.s globin synthesis (.alpha./.beta.s ratio) of 0.5, indicating the presence of an .alpha.-thalassemia gene. mRNA isolated from the bone marrow of the patient was translated in the wheat germ cell-free system, and the globin synthesized had an .alpha./.beta.s ratio of 0.7. The hemolysate prepared from incubated bone marrow cells was fractionated on a Sephadex G100 column. There was a peak of radioactivity that eluted after the Hb peak. When this pooled peak was analyzed by CMC [CM cellulose] chromatography, the .alpha./.beta.s ratio was 0.9. These globin intermediates, probably dimers, may have contributed to the hemolysis in this patient.This publication has 8 references indexed in Scilit:
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