Transcription factors Fli1 and EKLF in the differentiation of megakaryocytic and erythroid progenitor in 5q- syndrome and in Diamond-Blackfan anemia
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2013 |
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| Erschienen: |
2013 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:92 |
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| Enthalten in: |
Annals of hematology - 92(2013), 1 vom: 01. Jan., Seite 11-8 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Neuwirtova, Radana [VerfasserIn] |
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| Links: |
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| Themen: |
Erythroid Kruppel-like factor |
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| Anmerkungen: |
Date Completed 14.03.2013 Date Revised 19.11.2015 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1007/s00277-012-1568-1 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM22091026X |
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| 100 | 1 | |a Neuwirtova, Radana |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Transcription factors Fli1 and EKLF in the differentiation of megakaryocytic and erythroid progenitor in 5q- syndrome and in Diamond-Blackfan anemia |
| 264 | 1 | |c 2013 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 14.03.2013 | ||
| 500 | |a Date Revised 19.11.2015 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 7 | |a FLI1 protein, human |2 NLM | |
| 650 | 7 | |a Kruppel-Like Transcription Factors |2 NLM | |
| 650 | 7 | |a Proto-Oncogene Protein c-fli-1 |2 NLM | |
| 650 | 7 | |a RNA, Messenger |2 NLM | |
| 650 | 7 | |a Ribosomal Proteins |2 NLM | |
| 650 | 7 | |a erythroid Kruppel-like factor |2 NLM | |
| 700 | 1 | |a Fuchs, Ota |e verfasserin |4 aut | |
| 700 | 1 | |a Holicka, Monika |e verfasserin |4 aut | |
| 700 | 1 | |a Vostry, Martin |e verfasserin |4 aut | |
| 700 | 1 | |a Kostecka, Arnost |e verfasserin |4 aut | |
| 700 | 1 | |a Hajkova, Hana |e verfasserin |4 aut | |
| 700 | 1 | |a Jonasova, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Cermak, Jaroslav |e verfasserin |4 aut | |
| 700 | 1 | |a Cmejla, Radek |e verfasserin |4 aut | |
| 700 | 1 | |a Pospisilova, Dagmar |e verfasserin |4 aut | |
| 700 | 1 | |a Belickova, Monika |e verfasserin |4 aut | |
| 700 | 1 | |a Siskova, Magda |e verfasserin |4 aut | |
| 700 | 1 | |a Hochova, Ivana |e verfasserin |4 aut | |
| 700 | 1 | |a Vondrakova, Jana |e verfasserin |4 aut | |
| 700 | 1 | |a Sponerova, Dana |e verfasserin |4 aut | |
| 700 | 1 | |a Kadlckova, Eva |e verfasserin |4 aut | |
| 700 | 1 | |a Novakova, Ludmila |e verfasserin |4 aut | |
| 700 | 1 | |a Brezinova, Jana |e verfasserin |4 aut | |
| 700 | 1 | |a Michalova, Kyra |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Annals of hematology |d 1991 |g 92(2013), 1 vom: 01. Jan., Seite 11-8 |w (DE-627)NLM012918768 |x 1432-0584 |7 nnns |
| 773 | 1 | 8 | |g volume:92 |g year:2013 |g number:1 |g day:01 |g month:01 |g pages:11-8 |
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