早期恶性骨硬化症中的胎儿血红蛋白
Fetal haemoglobin in early malignant osteopetrosis.
作者信息
Schilirò G, Musumeci S, Pizzarelli G, Russo A, Marinucci M, Tentori L, Russo G
出版信息
Br J Haematol. 1978 Mar;38(3):339-44. doi: 10.1111/j.1365-2141.1978.tb01052.x.
Abstract
The characteristics of the fetal haemoglobin (HbF) in two children with osteopetrosis and high levels of HbF have been studied. The structural analysis of the gamma chains demonstrated a fetal Ggamma/Agamma ratio. HbF was distributed inside only 30% of the peripheral red blood cells. In vitro globin chain synthesis studies showed that there was balanced globin chain production, despite the increased level of HbF.
摘要
对两名患有骨质石化症且胎儿血红蛋白（HbF）水平较高的儿童的胎儿血红蛋白特征进行了研究。γ链的结构分析显示出胎儿Gγ/Aγ比例。HbF仅分布于30%的外周红细胞内。体外珠蛋白链合成研究表明，尽管HbF水平升高，但珠蛋白链的产生是平衡的。
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