Costa F F, Zago M A, Bottura C
Department of Clinical Medicine, School of Medicine, Ribeirão Preto, Brazil.
Birth Defects Orig Artic Ser. 1987;23(5A):157-61.
Globin-chain synthesis was studied in the peripheral blood and bone marrow of eight beta-thalassemia heterozygotes. Whole cell globin was prepared immediately after the labeling experiment. Chain separation of the bone marrow globin of each case was carried out with both the original material and after filtration on Sephadex G 100. The beta/alpha ratios obtained were (mean +/- SD): 0.46 +/- 0.05 in the peripheral blood, 0.61 +/- 0.06 in the unfiltered bone marrow globin, and 0.52 +/- 0.05 in the bone marrow globin after gel filtration. The results show that beta-thalassemia heterozygotes have a similar beta-chain deficiency in reticulocytes and bone marrow cells, provided whole cell globin is used, which avoids the removal of free alpha-chains.
对8名β地中海贫血杂合子的外周血和骨髓中的珠蛋白链合成进行了研究。标记实验结束后立即制备全细胞珠蛋白。对每个病例的骨髓珠蛋白，分别用原始材料以及经Sephadex G 100过滤后的材料进行链分离。得到的β/α比值为（平均值±标准差）：外周血中为0.46±0.05，未过滤的骨髓珠蛋白中为0.61±0.06，凝胶过滤后的骨髓珠蛋白中为0.52±0.05。结果表明，如果使用全细胞珠蛋白，避免游离α链的去除，β地中海贫血杂合子的网织红细胞和骨髓细胞中存在相似的β链缺陷。
