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Myelofibrosis: molecular and cell biological aspects
A subset of myeloproliferative disorders (MPN) and myelodyplastic syndromes (MDS) evolves to fibrosis of the bone marrow associated with haematopoietic insufficiency. We have been interested in chemokines involved in fibrogenesis within the bone marrow. Besides TGFβ we could identify a number of add...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3368793/ https://www.ncbi.nlm.nih.gov/pubmed/23259436 http://dx.doi.org/10.1186/1755-1536-5-S1-S21 |
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author | Kreipe, Hans Büsche, Guntram Bock, Oliver Hussein, Kais |
author_facet | Kreipe, Hans Büsche, Guntram Bock, Oliver Hussein, Kais |
author_sort | Kreipe, Hans |
collection | PubMed |
description | A subset of myeloproliferative disorders (MPN) and myelodyplastic syndromes (MDS) evolves to fibrosis of the bone marrow associated with haematopoietic insufficiency. We have been interested in chemokines involved in fibrogenesis within the bone marrow. Besides TGFβ we could identify a number of additional mediators including osteoprotegerin and bone morphogenic proteins. In MPN JAK2 or MPL mutation are not linked to the propensity for bone marrow fibrosis. The hypothesis that an increased intramedullary decay of megakaryocytes undergoing appotosis takes place within the marrow, thus liberating fibrogenic cytokines, could not be confirmed. On the contrary, megakaryocytes in primary fibrosis revealed low expression of proapoptotic genes such as BNIP3. Interestingly, BNIP 3 expression was down regulated in megakaryocytic cell lines kept in hypoxic conditions. Furthermore, expression arrays revealed hypoxia inducible genes to be up-regulated in primary myelofibrosis. Fibrotic MPN are characterized by aberrant proplatelet formation which represent cytoplasmic pseudopodia and normally extend into the sinus. In fibrotic MPN orientation of proplatelet growth appears to be disturbed, which could lead to an aberrant deposition of platelets in the marrow with consecutive liberation of fibrogenic cytokines. |
format | Online Article Text |
id | pubmed-3368793 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-33687932012-06-07 Myelofibrosis: molecular and cell biological aspects Kreipe, Hans Büsche, Guntram Bock, Oliver Hussein, Kais Fibrogenesis Tissue Repair Proceedings A subset of myeloproliferative disorders (MPN) and myelodyplastic syndromes (MDS) evolves to fibrosis of the bone marrow associated with haematopoietic insufficiency. We have been interested in chemokines involved in fibrogenesis within the bone marrow. Besides TGFβ we could identify a number of additional mediators including osteoprotegerin and bone morphogenic proteins. In MPN JAK2 or MPL mutation are not linked to the propensity for bone marrow fibrosis. The hypothesis that an increased intramedullary decay of megakaryocytes undergoing appotosis takes place within the marrow, thus liberating fibrogenic cytokines, could not be confirmed. On the contrary, megakaryocytes in primary fibrosis revealed low expression of proapoptotic genes such as BNIP3. Interestingly, BNIP 3 expression was down regulated in megakaryocytic cell lines kept in hypoxic conditions. Furthermore, expression arrays revealed hypoxia inducible genes to be up-regulated in primary myelofibrosis. Fibrotic MPN are characterized by aberrant proplatelet formation which represent cytoplasmic pseudopodia and normally extend into the sinus. In fibrotic MPN orientation of proplatelet growth appears to be disturbed, which could lead to an aberrant deposition of platelets in the marrow with consecutive liberation of fibrogenic cytokines. BioMed Central 2012-06-06 /pmc/articles/PMC3368793/ /pubmed/23259436 http://dx.doi.org/10.1186/1755-1536-5-S1-S21 Text en Copyright ©2012 Kreipe et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Proceedings Kreipe, Hans Büsche, Guntram Bock, Oliver Hussein, Kais Myelofibrosis: molecular and cell biological aspects |
title | Myelofibrosis: molecular and cell biological aspects |
title_full | Myelofibrosis: molecular and cell biological aspects |
title_fullStr | Myelofibrosis: molecular and cell biological aspects |
title_full_unstemmed | Myelofibrosis: molecular and cell biological aspects |
title_short | Myelofibrosis: molecular and cell biological aspects |
title_sort | myelofibrosis: molecular and cell biological aspects |
topic | Proceedings |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3368793/ https://www.ncbi.nlm.nih.gov/pubmed/23259436 http://dx.doi.org/10.1186/1755-1536-5-S1-S21 |
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