Alter B P, Schofield J M, He L Y, Weinberg R S
Polly Annenberg Levee Hematology Center, Department of Medicine, Mount Sinai School of Medicine New York, NY 10029.
Adv Exp Med Biol. 1989;271:95-102. doi: 10.1007/978-1-4613-0623-8_11.
It is clear that in vitro hemin increases the number of blood BFU-E derived colonies from normal donors. This occurs with sickle donors as well, despite the increased levels of hemin in vivo in these patients. The effect of hemin on relative gamma globin synthesis is inconsistent, however. In a few cases, delayed addition of hemin led to increased gamma globin synthesis. In time course studies of cultures from normal donors, hemin added on day 0 shifted the day of peak colony number from 13-14 to 16-20 days. The temporal decline in gamma globin synthesis was not altered. In cultures from sickle donors we found that the time for maximal colony number was later than in normals, occurring at 16-20 days even without hemin, and was not further delayed by hemin. The relative proportion of gamma globin synthesis was higher on day 14 in the sickle than the normal cultures, and the temporal decline was somewhat slowed in the sickle cultures by hemin. The elevated gamma synthesis and the later time for peak colony growth in the sickle cultures suggest that the erythroid progenitors in the blood of the sickle patients are less mature than those from normal individuals. There are several possible explanations for the detection of increased numbers of colonies in cultures containing hemin. Hemin may delay the final maturation of erythroblasts within erythroid colonies, thus shifting the time of maximal growth. It may also increase the extent of final maturation, leading to more complete hemoglobinization of the erythroblasts within the colonies, and thus increasing the number of colonies that are eventually recognized as erythroid.(ABSTRACT TRUNCATED AT 250 WORDS)
显然，体外血红素可增加正常供者血液中BFU-E衍生集落的数量。镰状细胞病供者也会出现这种情况，尽管这些患者体内的血红素水平升高。然而，血红素对相对γ珠蛋白合成的影响并不一致。在少数情况下，延迟添加血红素会导致γ珠蛋白合成增加。在对正常供者培养物的时间进程研究中，第0天添加血红素会使集落数量峰值出现的时间从13 - 14天推迟到16 - 20天。γ珠蛋白合成的时间性下降并未改变。在镰状细胞病供者的培养物中，我们发现达到最大集落数量的时间比正常供者晚，即使不添加血红素也在16 - 20天出现，添加血红素后也不会进一步延迟。镰状细胞病培养物中γ珠蛋白合成的相对比例在第14天高于正常培养物，并且血红素使镰状细胞病培养物中的时间性下降有所减缓。镰状细胞病培养物中γ珠蛋白合成增加以及集落生长峰值出现时间较晚，这表明镰状细胞病患者血液中的红系祖细胞比正常个体的更不成熟。对于在含血红素的培养物中检测到集落数量增加有几种可能的解释。血红素可能会延迟红系集落内成红细胞的最终成熟，从而改变最大生长时间。它也可能增加最终成熟的程度，导致集落内成红细胞的血红蛋白化更完全，从而增加最终被识别为红系的集落数量。（摘要截选至250字）
