Steinberg M H, Coleman M, Dreiling B
Br J Haematol. 1976 Sep;34(1):55-60. doi: 10.1111/j.1365-2141.1976.tb00173.x.
Globin biosynthesis was studied in both erythroid precursors and reticulocytes of three individuals with heterozygous alpha-thalassaemia. In contrast to the finding of equal or nearly equal alpha and beta chain synthesis in the marrow of patients with heterozygous beta-thalassaemia previously examined, our studies showed equal degrees of unbalanced globin synthesis in both reticulocytes and nucleated-erythroid cells of alpha-thalassaemia heterozygotes. Greater stability and less susceptibility to proteolysis of the excess beta-chain formed in alpha-thalassaemia may explain our findings.
对三名杂合子α地中海贫血患者的红系前体细胞和网织红细胞中的珠蛋白生物合成进行了研究。与之前检测的杂合子β地中海贫血患者骨髓中α链和β链合成相等或几乎相等的结果相反,我们的研究表明,α地中海贫血杂合子的网织红细胞和有核红细胞中均存在程度相同的珠蛋白合成失衡。α地中海贫血中形成的过量β链具有更高的稳定性和更低的蛋白水解敏感性,这可能解释了我们的研究结果。