Abstract |
A 15-yr-old black male with homozygous sickle cell disease was severely growth retarded and had a chronic hemolytic anemia requiring transfusions. Globin chain synthetic studies of both peripheral blood reticulocytes and bone marrow cells revealed a ratio of alpha to betas globin synthesis (alpha/betas ratio) of 0.5, indicating the presence of an alpha-thalassemia gene. Messenger RNA isolated from the bone marrow of the patient was translated in the wheat germ cell-free system, and the globin synthesized had an alpha/betas ratio of 0.7. The hemolysate prepared from incubated bone marrow cells was fractionated on a Sephadex G100 column. The results showed that there was a peak of radioactivity that eluted after the hemoglobin peak. When this pooled peak was analyzed by CMC chromatography, the alpha/betas ratio was 0.9. These globin intermediates, probably dimers, may have contributed to the hemolysis in this patient.
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Authors | C Natta |
Journal | Blood
(Blood)
Vol. 51
Issue 6
Pg. 1163-8
(Jun 1978)
ISSN: 0006-4971 [Print] United States |
PMID | 647121
(Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
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Topics |
- Adolescent
- Anemia, Sickle Cell
(blood)
- Bone Marrow
(metabolism)
- Chromatography, Gel
- Genes
- Globins
(biosynthesis)
- Humans
- Male
- RNA, Messenger
- Thalassemia
(genetics)
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