McGuckin C P, Uhr M R, Liu W M, Gordon-Smith E C
Department of Cellular and Molecular Sciences, St George's Hospital Medical School, London, UK.
Eur J Haematol. 1996 Jul;57(1):72-8. doi: 10.1111/j.1600-0609.1996.tb00493.x.
Stem cell factor (SCF) is the ligand for the dimeric c-kit tyrosine kinase receptor. Binding of SCF to c-kit is a crucial element in the developmental stimulus of late stem cells and early progenitor cells. In the erythroid lineage the SCF stimulus is important not only for proliferation and differentiation, but is also known to enhance later haemoglobin production. In an earlier report we described a rapid non-radioactive technique using the extended ester-attached labelled SCF protein itself for detecting c-kit expression in marrow and peripheral blood mononuclear populations. In the present study we have taken this a step further to analyse c-kit expression in developing erythroid cells in vitro, principally using normal donor samples. This was designed for use as a foundation for the comparison of haematological disorders. In this case we tested 4 patients with the congenital disorder of erythropoiesis, Diamond-Blackfan anaemia (DBA), finding that in all cases DBA c-kit expression was elevated over normal, in 1 case as high as 348% of the normal average. This may be indicative of the reduced state of progenitor development in these patients. These results show that the described technique is beneficial for analysis in the stem and progenitor compartment.
干细胞因子（SCF）是二聚体c-kit酪氨酸激酶受体的配体。SCF与c-kit的结合是晚期干细胞和早期祖细胞发育刺激中的关键要素。在红系谱系中，SCF刺激不仅对增殖和分化很重要，而且已知还能增强后期血红蛋白的产生。在早期的一份报告中，我们描述了一种快速非放射性技术，该技术使用与酯相连的标记SCF蛋白本身来检测骨髓和外周血单个核细胞群体中的c-kit表达。在本研究中，我们进一步采取措施，主要使用正常供体样本，分析体外发育中的红系细胞中的c-kit表达。这旨在作为比较血液系统疾病的基础。在这种情况下，我们检测了4例患有先天性红细胞生成障碍——先天性纯红细胞再生障碍性贫血（DBA）的患者，发现所有病例中DBA的c-kit表达均高于正常水平，其中1例高达正常平均值的348%。这可能表明这些患者中祖细胞发育状态降低。这些结果表明，所描述的技术有利于在干细胞和祖细胞区室中进行分析。
