Horiuchi K, Osterhout M L, Ohene-Frempong K
Children's Hospital of Philadelphia, Department of Pediatrics University of Pennsylvania School of Medicine 19104, USA.
Biochem Biophys Res Commun. 1995 Dec 26;217(3):924-30. doi: 10.1006/bbrc.1995.2859.
Fetal hemoglobin, Hb F, is known to be an important factor for clinical course of sickle cell disease, as it suppresses polymerization of sickle hemoglobin. To investigate the effect of Hb F on the survival of sickle reticulocytes (young red cells) in circulation, Hb F levels in individual reticulocytes and mature erythrocytes were quantified via fluorescence image cytometry. We first examined unfractionated SS cells from 3 patients with different Hb F levels, and found that Hb F levels in reticulocyte populations were always lower than those in erythrocyte populations. This suggests that subsets of reticulocytes with lower Hb F levels are removed during maturation while those with higher Hb F levels tend to survive to become erythrocytes. The distribution of Hb F in reticulocytes was different among these patients and seems to strongly affect the survival of F-reticulocytes. We also analyzed density-separated fractions, and found that Hb F levels in reticulocytes found in the densest fraction were lower than those in lighter fractions. This suggests that reticulocytes with lower Hb F levels are susceptible to quick dehydration within their maturation period (1-2 days) in circulation.
胎儿血红蛋白（Hb F）已知是镰状细胞病临床病程的一个重要因素，因为它能抑制镰状血红蛋白的聚合。为了研究Hb F对循环中镰状网织红细胞（年轻红细胞）存活的影响，通过荧光图像细胞术对单个网织红细胞和成熟红细胞中的Hb F水平进行了定量分析。我们首先检查了3名不同Hb F水平患者的未分级SS细胞，发现网织红细胞群体中的Hb F水平总是低于红细胞群体中的Hb F水平。这表明，Hb F水平较低的网织红细胞亚群在成熟过程中被清除，而Hb F水平较高的网织红细胞亚群则倾向于存活下来成为红细胞。这些患者中网织红细胞内Hb F的分布各不相同，似乎对F网织红细胞的存活有强烈影响。我们还分析了密度分离的组分，发现密度最大的组分中网织红细胞的Hb F水平低于较轻组分中的水平。这表明，Hb F水平较低的网织红细胞在其循环成熟阶段（1 - 2天）内易快速脱水。
