Globin-chain synthesis in Hb H disease: the activity of red cell precursors and their mRNA

Globin-chain synthesis was determined in bone marrow and blood of four patients with Hb H disease and in a cell-free system supplemented with poly(A)-rich RNA from the patients. The relative synthesis of alpha-globin ranged between 0.36 and 0.49 in erythroid precursors and between 0.09 and 0.28 in r...

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Bibliographic Details
Published inIsrael journal of medical sciences Vol. 14; no. 11; p. 1102
Main Authors Kirschmann, C, Lupovitz, Z, Steinherz, M, Zaizov, R
Format Journal Article
LanguageEnglish
Published Israel 01.11.1978
Subjects
Bone Marrow - metabolism
Globins - biosynthesis
Hemoglobin H
Hemoglobinopathies - blood
Hemoglobinopathies - metabolism
Hemoglobins, Abnormal
Humans
Reticulocytes - metabolism
RNA, Messenger - blood
RNA, Messenger - metabolism
Thalassemia - blood
Thalassemia - metabolism
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Summary:Globin-chain synthesis was determined in bone marrow and blood of four patients with Hb H disease and in a cell-free system supplemented with poly(A)-rich RNA from the patients. The relative synthesis of alpha-globin ranged between 0.36 and 0.49 in erythroid precursors and between 0.09 and 0.28 in reticulocytes. The relative quantity of biologically active alpha-globin mRNA in poly(A)-rich RNA from bone marrow and blood of these patients ranged between 0.09 and 0.21. The findings suggest the operation, within alpha-thalassemic bone marrow cells, of a translational control mechanism and/or the instability of alpha-globin mRNA.
ISSN:0021-2180