Search Results - "van Strien, Paulina M.H."

Malignant Transformation Involving CXXC4 Mutations Identified in a Leukemic Progression Model of Severe Congenital Neutropenia by Olofsen, Patricia A., Fatrai, Szabolcs, van Strien, Paulina M.H., Obenauer, Julia C., de Looper, Hans W.J., Hoogenboezem, Remco M., Erpelinck-Verschueren, Claudia A.J., Vermeulen, Michael P.W.M., Roovers, Onno, Haferlach, Torsten, Jansen, Joop H., Ghazvini, Mehrnaz, Bindels, Eric M.J., Schneider, Rebekka K., de Pater, Emma M., Touw, Ivo P.

“…Severe congenital neutropenia (SCN) patients treated with CSF3/G-CSF to alleviate neutropenia frequently develop acute myeloid leukemia (AML). A common pattern…”
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Mesenchymal Inflammation Drives Genotoxic Stress in Hematopoietic Stem Cells and Predicts Disease Evolution in Human Pre-leukemia by Zambetti, Noemi A., Ping, Zhen, Chen, Si, Kenswil, Keane J.G., Mylona, Maria A., Sanders, Mathijs A., Hoogenboezem, Remco M., Bindels, Eric M.J., Adisty, Maria N., Van Strien, Paulina M.H., van der Leije, Cindy S., Westers, Theresia M., Cremers, Eline M.P., Milanese, Chiara, Mastroberardino, Pier G., van Leeuwen, Johannes P.T.M., van der Eerden, Bram C.J., Touw, Ivo P., Kuijpers, Taco W., Kanaar, Roland, van de Loosdrecht, Arjan A., Vogl, Thomas, Raaijmakers, Marc H.G.P.

“…Mesenchymal niche cells may drive tissue failure and malignant transformation in the hematopoietic system, but the underlying molecular mechanisms and…”
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Truncated CSF3 receptors induce pro‐inflammatory responses in severe congenital neutropenia by Olofsen, Patricia A., Bosch, Dennis A., Looper, Hans W. J., Strien, Paulina M. H., Hoogenboezem, Remco M., Roovers, Onno, Velden, Vincent H. J., Bindels, Eric M. J., De Pater, Emma M., Touw, Ivo P.

“…Summary Severe congenital neutropenia (SCN) patients are prone to develop myelodysplastic syndrome (MDS) or acute myeloid leukaemia (AML). Leukaemic…”
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An autonomous CEBPA enhancer specific for myeloid-lineage priming and neutrophilic differentiation by Avellino, Roberto, Havermans, Marije, Erpelinck, Claudia, Sanders, Mathijs A., Hoogenboezem, Remco, van de Werken, Harmen J.G., Rombouts, Elwin, van Lom, Kirsten, van Strien, Paulina M.H., Gebhard, Claudia, Rehli, Michael, Pimanda, John, Beck, Dominik, Erkeland, Stefan, Kuiken, Thijs, de Looper, Hans, Gröschel, Stefan, Touw, Ivo, Bindels, Eric, Delwel, Ruud

“…Neutrophilic differentiation is dependent on CCAAT enhancer-binding protein α (C/EBPα), a transcription factor expressed in multiple organs including the bone…”
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Myeloid cells promote interferon signaling-associated deterioration of the hematopoietic system by Feyen, Jacqueline, Ping, Zhen, Chen, Lanpeng, van Dijk, Claire, van Tienhoven, Tim V. D., van Strien, Paulina M. H., Hoogenboezem, Remco M., Wevers, Michiel J. W., Sanders, Mathijs A., Touw, Ivo P., Raaijmakers, Marc H. G. P.

“…Innate and adaptive immune cells participate in the homeostatic regulation of hematopoietic stem cells (HSCs). Here, we interrogate the contribution of myeloid…”
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Gli1+ Mesenchymal Stromal Cells Are a Key Driver of Bone Marrow Fibrosis and an Important Cellular Therapeutic Target by Schneider, Rebekka K., Mullally, Ann, Dugourd, Aurelien, Peisker, Fabian, Hoogenboezem, Remco, Van Strien, Paulina M.H., Bindels, Eric M., Heckl, Dirk, Büsche, Guntram, Fleck, David, Müller-Newen, Gerhard, Wongboonsin, Janewit, Ventura Ferreira, Monica, Puelles, Victor G., Saez-Rodriguez, Julio, Ebert, Benjamin L., Humphreys, Benjamin D., Kramann, Rafael

“…Bone marrow fibrosis (BMF) develops in various hematological and non-hematological conditions and is a central pathological feature of myelofibrosis. Effective…”
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Gli1+ Mesenchymal Stromal Cells Are a Key Driver of Bone Marrow Fibrosis and an Important Cellular Therapeutic Target by Schneider, Rebekka K., Mullally, Ann, Dugourd, Aurelien, Peisker, Fabian, Hoogenboezem, Remco, Van Strien, Paulina M.H., Bindels, Eric M., Heckl, Dirk, Büsche, Guntram, Fleck, David, Müller-Newen, Gerhard, Wongboonsin, Janewit, Ventura Ferreira, Monica, Puelles, Victor G., Saez-Rodriguez, Julio, Ebert, Benjamin L., Humphreys, Benjamin D., Kramann, Rafael

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ICL-induced miR139-3p and miR199a-3p have opposite roles in hematopoietic cell expansion and leukemic transformation by Alemdehy, Mir Farshid, Haanstra, Jurgen R., de Looper, Hans W.J., van Strien, Paulina M.H., Verhagen-Oldenampsen, Judith, Caljouw, Yvette, Sanders, Mathijs A., Hoogenboezem, Remco, de Ru, Arnoud H., Janssen, George M.C., Smetsers, Stephanie E., Bierings, Marc B., van Veelen, Peter A., von Lindern, Marieke, Touw, Ivo P., Erkeland, Stefan J.

“…Interstrand crosslinks (ICLs) are toxic DNA lesions that cause severe genomic damage during replication, especially in Fanconi anemia pathway-deficient cells…”
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Deficiency of the ribosome biogenesis gene Sbds in hematopoietic stem and progenitor cells causes neutropenia in mice by attenuating lineage progression in myelocytes by Zambetti, Noemi A, Bindels, Eric M J, Van Strien, Paulina M H, Valkhof, Marijke G, Adisty, Maria N, Hoogenboezem, Remco M, Sanders, Mathijs A, Rommens, Johanna M, Touw, Ivo P, Raaijmakers, Marc H G P

“…Shwachman-Diamond syndrome is a congenital bone marrow failure disorder characterized by debilitating neutropenia. The disease is associated with…”
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Essential role for Gata2 in modulating lineage output from hematopoietic stem cells in zebrafish by Gioacchino, Emanuele, Koyunlar, Cansu, Zink, Joke, de Looper, Hans, de Jong, Madelon, Dobrzycki, Tomasz, Mahony, Christopher B., Hoogenboezem, Remco, Bosch, Dennis, van Strien, Paulina M.H., van Royen, Martin E., French, Pim J., Bindels, Eric, Gussinklo, Kirsten J., Monteiro, Rui, Touw, Ivo P., de Pater, Emma

“…•Gata2b is required for embryonic HSPC expansion, but not HSPC generation in zebrafish.•Gata2b plays an instructive role in the lineage output of HSPCs in…”
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RUNX1 TAD and RHD Mutations Found in SCN/AML Differentially Affect HSPC Expansion in Conjunction with Truncated CSF3 Receptors by Obenauer, Julia C., van Strien, Paulina M.H., Hoogenboezem, Remco M., van den Heuvel-Eibrink, Marry M., Touw, Ivo P.

“…Severe congenital neutropenia (SCN) is characterized by a maturation arrest at the promyelocyte stage and consequently a severe reduction of peripheral…”
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Aneuploidy and Overexpression of Ki67 and p53 as Markers for Neoplastic Progression in Barrett's Esophagus: A Case-Control Study by SIKKEMA, Marjolein, KERKHOF, Marjon, STEYERBERG, Ewout W, KUSTERS, Johannes G, VAN STRIEN, Paulina M. H, LOOMAN, Caspar WN, VAN DEKKEN, Herman, SIERSEMA, Peter D, KUIPERS, Ernst J

“…Surveillance of patients with Barrett's esophagus (BE) aims at early detection and treatment of neoplastic changes, particularly esophageal adenocarcinoma…”
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Inflammatory Niche Signalling Drives Genotoxic Stress in Hematopoietic Stem Cells and Predicts Leukemic Evolution in Human Leukemia Predisposition Syndromes by Chen, Si, Zambetti, Noemi A., Ping, Zhen, Kenswil, Keane, Mylona, Maria, Sanders, Mathijs A., Hoogenboezem, Remco, Bindels, Eric, Adisty, Maria, van Strien, Paulina M.H., van der Leije, Cindy, Westers, Theresia M, Cremers, Eline MP, Milanese, Chiara, Mastroberardino, Pier, van Leeuwen, Johannes, Van der Eerden, Bram, Touw, Ivo, Taco, Kuijpers, Kanaar, Roland, van der Loosdrecht, Arjan, Vogl, Thomas, Raaijmakers, Marc H.G.P.

“…Primary alterations of the mesenchymal niche can induce myelodysplasia and acute myeloid leukemia in mouse models, introducing a concept of niche-driven…”
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Deletion of Sbds from Hematopoietic Progenitors Causes Neutropenia in a Mouse Model of Shwachman-Diamond Syndrome By Specifically Blocking Myeloid Lineage Progression at Late Differentiation Stages by Zambetti, Noemi A., Van Strien, Paulina M.H., Valkhof, Marijke G., Adisty, Maria N., Bindels, Eric M.J., Hoogenboezem, Remco M., Sanders, Mathijs A., Rommens, Johanna M., Touw, Ivo P., Raaijmakers, Marc H.G.P.

“…Shwachman-Diamond Syndrome (SDS) is a congenital bone marrow failure disorder characterized by neutropenia and predisposition to leukemia. SDS is associated…”
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PML-controlled responses in severe congenital neutropenia with ELANE-misfolding mutations by Olofsen, Patricia A., Bosch, Dennis A., Roovers, Onno, van Strien, Paulina M.H., de Looper, Hans W.J., Hoogenboezem, Remco M., Barnhoorn, Sander, Mastroberardino, Pier G., Ghazvini, Mehrnaz, van der Velden, Vincent H.J., Bindels, Eric M.J., de Pater, Emma M., Touw, Ivo P.

“…Mutations in ELANE cause severe congenital neutropenia (SCN), but how they affect neutrophil production and contribute to leukemia predisposition is unknown…”
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Hematopoietic Cell Autonomous Disruption of Hematopoiesis in a Germline Loss‐of‐function Mouse Model of RUNX1‐FPD by Ernst, Martijn P. T., Pronk, Eline, Dijk, Claire, Strien, Paulina M. H., Tienhoven, Tim V. D., Wevers, Michiel J. W., Sanders, Mathijs A., Bindels, Eric M. J., Speck, Nancy A., Raaijmakers, Marc H. G. P.

“…RUNX1 familial platelet disorder (RUNX1‐FPD) is a hematopoietic disorder caused by germline loss‐of‐function mutations in the RUNX1 gene and characterized by…”
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Lack of splice factor and cohesin complex mutations in pediatric myelodysplastic syndrome by Obenauer, Julia C, Kavelaars, François G, Sanders, Mathijs A, Hoogenboezem, Remco M, de Vries, Andrica C H, van Strien, Paulina M H, de Haas, Valerie, Locatelli, Franco, Hasle, Henrik, Valk, Peter J M, Touw, Ivo P, van den Heuvel-Eibrink, Marry M

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Loss of Ercc1 Results in a Time- and Dose-Dependent Reduction of Proliferating Early Hematopoietic Progenitors by Verhagen-Oldenampsen, Judith H. E., Haanstra, Jurgen R., van Strien, Paulina M. H., Valkhof, Marijke, Touw, Ivo P., von Lindern, Marieke

“…The endonuclease complex Ercc1/Xpf is involved in interstrand crosslink repair and functions downstream of the Fanconi pathway. Loss of Ercc1 causes…”
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