Pellagatti Andrea, Marafioti Teresa, Paterson Jennifer C, Malcovati Luca, Della Porta Matteo G, Jädersten Martin, Pushkaran Beena, George Tracy I, Arber Daniel A, Killick Sally, Giagounidis Aristoteles, Hellström-Lindberg Eva, Cazzola Mario, Wainscoat James S, Boultwood Jacqueline
LRF Molecular Haematology Unit, NDCLS, John Radcliffe Hospital, Oxford, UK.
Br J Haematol. 2009 Jun;146(1):86-90. doi: 10.1111/j.1365-2141.2009.07720.x. Epub 2009 May 5.
Lymphoid enhancer-binding factor 1 (LEF1) is a neutrophilic granulopoiesis regulator whose absence is critical in congenital neutropenia. We have shown LEF1 downregulation in the CD34(+) cells of the majority of myelodysplastic syndromes (MDS) patients. LEF1 was the most significant differentially expressed gene between early and advanced MDS. Marked LEF1 downregulation was found in 27/32 patients with advanced MDS and in 6/35 patients with early MDS, and was associated with neutropenia. Downregulation of LEF1 mRNA was reflected at the protein level. Immunostaining for CD34/LEF1 may represent a marker of advanced MDS. LEF1 may play a role in the defective maturation of myeloid progenitors in MDS.
淋巴细胞增强因子1（LEF1）是一种嗜中性粒细胞生成调节因子，其缺失在先天性中性粒细胞减少症中至关重要。我们已经证明，大多数骨髓增生异常综合征（MDS）患者的CD34(+)细胞中LEF1表达下调。LEF1是早期和晚期MDS之间差异表达最显著的基因。在32例晚期MDS患者中有27例以及35例早期MDS患者中有6例发现LEF1明显下调，且与中性粒细胞减少有关。LEF1 mRNA的下调在蛋白质水平也有体现。CD34/LEF1免疫染色可能代表晚期MDS的一个标志物。LEF1可能在MDS中髓系祖细胞的成熟缺陷中起作用。
