Kirschmann C, Lupovitz Z, Steinherz M, Zaizov R
Isr J Med Sci. 1978 Nov;14(11):1102-6.
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.
在4例血红蛋白H病患者的骨髓和血液中，以及在补充了来自这些患者的富含多聚腺苷酸（poly(A)）RNA的无细胞系统中，测定了珠蛋白链的合成情况。α-珠蛋白在红系前体细胞中的相对合成量在0.36至0.49之间，在网织红细胞中的相对合成量在0.09至0.28之间。这些患者骨髓和血液中富含多聚腺苷酸RNA的生物活性α-珠蛋白mRNA的相对量在0.09至0.21之间。这些发现提示，在α地中海贫血骨髓细胞内存在翻译控制机制和/或α-珠蛋白mRNA的不稳定性。
