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地中海贫血综合征的生化特征是控制血红蛋白中一个珠蛋白链的合成的信使核糖核酸有遗传缺陷,临床特征是具有不同程度的溶血性贫血,黄疸和脾肿大。β地中海贫血时,β链合成降低,而α链合成率保持不变。因此,不稳定的α链相对过剩,沉淀为细胞内的包涵
The biochemical characteristics of thalassemia syndrome are genetic defects in the synthesis of a messenger RNA that controls a globin chain in hemoglobin. The clinical features are hemolytic anemia, jaundice and splenomegaly with varying degrees of clinical features. β-thalassemia, β-chain synthesis decreases, while α-chain synthesis rates remain unchanged. As a result, the unstable α-chain is relatively excessively precipitated into intracellular inclusions