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 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.
D001853:Bone Marrow / Q000378:metabolism*; D005914:Globins / Q000096:biosynthesis*; D006447:Hemoglobin H; D006453:Hemoglobinopathies / Q000097:blood / Q000378:metabolism*; D006455:Hemoglobins, Abnormal; D006801:Humans; D012333:RNA, Messenger / Q000097:blood / Q000378:metabolism*; D012156:Reticulocytes / Q000378:metabolism*; D013789:Thalassemia / Q000097:blood / Q000378:metabolism
D006455:Hemoglobins, Abnormal; D012333:RNA, Messenger; D005914:Globins; D006447:Hemoglobin H