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Failure of the alpha-thalassemia gene to decrease the severity of sickle cell anemia
| Content Provider | Scilit |
|---|---|
| Author | Natta, C. |
| Copyright Year | 1978 |
| Description | 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. |
| Related Links | https://ashpublications.org/blood/article-pdf/51/6/1163/581491/1163.pdf |
| Ending Page | 1168 |
| Page Count | 6 |
| Starting Page | 1163 |
| DOI | 10.1182/blood.v51.6.1163.1163 |
| Journal | Blood |
| Issue Number | 6 |
| Volume Number | 51 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 1978-06-01 |
| Access Restriction | Open |
| Subject Keyword | Medical Laboratory Technology Bone Marrow Anemia Thalassemia Sickle Cell Globin Alpha/betas Ratio Journal: Blood (Vol- 86, Issue- 6) |
| Content Type | Text |
| Resource Type | Article |
