Transcription factors Fli1 and EKLF in the differentiation of megakaryocytic and erythroid progenitor in 5q- syndrome and in Diamond-Blackfan anemia
Language English Country Germany Media print-electronic
Document type Journal Article, Research Support, Non-U.S. Gov't
- MeSH
- Bone Marrow Cells metabolism MeSH
- Chromosome Deletion MeSH
- CpG Islands MeSH
- Anemia, Diamond-Blackfan genetics metabolism MeSH
- Child MeSH
- Adult MeSH
- Erythropoiesis genetics MeSH
- Transcription, Genetic MeSH
- Real-Time Polymerase Chain Reaction MeSH
- Leukocytes, Mononuclear metabolism MeSH
- Humans MeSH
- Chromosomes, Human, Pair 5 genetics metabolism MeSH
- Anemia, Macrocytic genetics metabolism MeSH
- RNA, Messenger biosynthesis blood MeSH
- Adolescent MeSH
- Young Adult MeSH
- Myelodysplastic Syndromes genetics metabolism MeSH
- Proto-Oncogene Protein c-fli-1 biosynthesis genetics physiology MeSH
- Ribosomal Proteins physiology MeSH
- Kruppel-Like Transcription Factors biosynthesis genetics physiology MeSH
- Thrombopoiesis genetics MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- erythroid Kruppel-like factor MeSH Browser
- FLI1 protein, human MeSH Browser
- RNA, Messenger MeSH
- Proto-Oncogene Protein c-fli-1 MeSH
- Ribosomal Proteins MeSH
- Kruppel-Like Transcription Factors MeSH
Friend leukemia virus integration 1 (Fli1) and erythroid Krüppel-like factor (EKLF) participate under experimental conditions in the differentiation of megakaryocytic and erythroid progenitor in cooperation with other transcription factors, cytokines, cytokine receptors, and microRNAs. Defective erythropoiesis with refractory anemia and effective megakaryopoiesis with normal or increased platelet count is typical for 5q- syndrome. We decided to evaluate the roles of EKLF and Fli1 in the pathogenesis of this syndrome and of another ribosomopathy, Diamond-Blackfan anemia (DBA). Fli1 and EKLF mRNA levels were examined in mononuclear blood and bone marrow cells from patients with 5q- syndrome, low-risk MDS patients with normal chromosome 5, DBA patients, and healthy controls. In 5q- syndrome, high Fli1 mRNA levels in the blood and bone marrow mononuclear cells were found. In DBA, Fli1 expression did not differ from the controls. EKLF mRNA level was significantly decreased in the blood and bone marrow of 5q- syndrome and in all DBA patients. We propose that the elevated Fli1 in 5q- syndrome protects megakaryocytic cells from ribosomal stress contrary to erythroid cells and contributes to effective though dysplastic megakaryopoiesis.
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