Wood W G, Stamatoyannopoulos G
Hemoglobin. 1976;1(2):135-51. doi: 10.3109/03630267608991676.
Globin chain synthesis was examined in erythroid cells of increasing maturity, fractionated from bone marrow of two patients with hemoglobin H disease and in one alpha thalassemia 1 heterozygote. In contrast to beta thalassemia where a gradient of alpha/beta chain ratios increasing with erythroid cell maturation is observed, in alpha thalassemia the alpha/beta chain ratio remains constant throughout maturation. This finding suggests that in alpha thalassemia there is no modification of the imbalance in globin chain synthesis either by increased alpha chain production or decreased beta chain synthesis in erythroid precursors. Furthermore, the constant alpha/beta ratio reflects a limited degree of beta chain destruction, indicating that the ability of the excess beta chains to associate into tetramers protects them from proteolytic digestion.
对两名血红蛋白H病患者和一名α地中海贫血1杂合子的骨髓中分离出的成熟度不断增加的红系细胞中的珠蛋白链合成进行了检测。与β地中海贫血不同，在β地中海贫血中观察到随着红系细胞成熟α/β链比例呈梯度增加，而在α地中海贫血中，α/β链比例在整个成熟过程中保持恒定。这一发现表明，在α地中海贫血中，红系前体细胞中珠蛋白链合成的失衡不会因α链产量增加或β链合成减少而改变。此外，恒定的α/β比例反映了β链破坏程度有限，表明过量的β链缔合成四聚体的能力保护它们免受蛋白水解消化。
