Marked downregulation of the granulopoiesis regulator LEF1 is associated with disease progression in the myelodysplastic syndromes.

Journal article

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.

Authors: Pellagatti, A; Marafioti, T; Paterson, J; Malcovati, L; Della Porta, MG

• Publication status: Published
• Journal: British journal of haematology
• Volume: 146
• Issue: 1
• Pages: 86-90
• Publication date: 2009-06-01
• DOI: 10.1111/j.1365-2141.2009.07720.x
• EISSN: 1365-2141
• ISSN: 0007-1048
• Language: English
• Keywords: Oligonucleotide Array Sequence Analysis; Case-Control Studies; Sequence Analysis, DNA; Myelodysplastic Syndromes; Gene Expression Profiling; Humans; Lymphoid Enhancer-Binding Factor 1; Disease Progression; Down-Regulation; RNA, Messenger; Immunohistochemistry; Reverse Transcriptase Polymerase Chain Reaction; Antigens, CD34; Neutropenia