Beat W. Schäfer
Boston Children's Hospital
GeneCancerCalcium-binding proteinFusion proteinInternal medicinePathologyMolecular biologyPAX3ChemistryImmunologyAlveolar rhabdomyosarcomaSarcomaRhabdomyosarcomaCancer researchGeneticsMedicineCell cultureTranscription factorBiologyCell biology
210Publications
71H-index
11.6kCitations
Publications 211
Newest
#1Melanie GeneraliH-Index: 4
Last. Elisa A. CasanovaH-Index: 7
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Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Fusion-positive RMS (FPRMS), expressing the PAX3/7-FOXO1, has a worse prognosis compared to the more common fusion-negative RMS (FNRMS). Although several studies reported hierarchical organization for FNRMS with the identification of cancer stem cells, the cellular organization of FPRMS is not yet clear. In this study we investigated the expression of key stem cell markers, developed a sphere assay, and investigated the s...
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#1Gabriele Manzella (Boston Children's Hospital)H-Index: 3
#2Devmini C Moonamale (Boston Children's Hospital)
Last. Beat W. Schäfer (Boston Children's Hospital)H-Index: 71
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First-line therapy for most pediatric sarcoma is based on chemotherapy in combination with radiotherapy and surgery. A significant number of patients experience drug resistance and development of relapsed tumors. Drugs that have the potential to re-sensitize relapsed tumor cells toward chemotherapy treatment are therefore of great clinical interest. Here, we used a drug profiling platform with PDX-derived primary rhabdomyosarcoma cells to screen a large drug library for compounds re-sensitizing ...
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#1Carla Regina (University Medical Center Freiburg)
#2Geoffroy Andriuex (University Medical Center Freiburg)
Last. Anton G. Henssen (Charité)H-Index: 13
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Background: Rhabdomyosarcomas (RMS) are phenotypically and functionally heterogeneous. Expression of the fusion oncogene PAX3:FOXO1 (P3F) was previously shown to differ between individual tumor cells and fluctuate over time. null Methods: In mouse Myf6Cre+/-,Pax3:Foxo1+/+,p53-/- RMS tumors, expression of P3F is directed by the Pax3 promoter and coupled to an eYFP fluorescent marker, which is activated as a second cistron downstream from an encephalomyocarditis virus-derived internal ribosome ent...
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#1Carla Regina (University Medical Center Freiburg)
#2Ebrahem Hamed (University Medical Center Freiburg)
Last. Ken Kikuchi (Kyoto Prefectural University of Medicine)H-Index: 14
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Rhabdomyosarcomas (RMS) are phenotypically and functionally heterogeneous. Both primary human RMS cultures and low-passage Myf6Cre,Pax3:Foxo1,p53 mouse RMS cell lines, which express the fusion oncoprotein Pax3:Foxo1 and lack the tumor suppressor Tp53 (Myf6Cre,Pax3:Foxo1,p53), exhibit marked heterogeneity in PAX3:FOXO1 (P3F) expression at the single cell level. In mouse RMS cells, P3F expression is directed by the Pax3 promoter and coupled to eYFP YFPlow/P3Flow mouse RMS cells included 87% G0/G1 ...
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#1Eva Brack (Boston Children's Hospital)H-Index: 2
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#1David O. Azorsa (Boston Children's Hospital)H-Index: 3
#2Peter K. Bode (UZH: University of Zurich)H-Index: 13
Last. Javed KhanH-Index: 27
view all 11 authors...
Alveolar Rhabdomyosarcoma (ARMS) is an aggressive pediatric cancer with about 80% of cases characterized by either a t(1;13)(p36;q14) or t(2;13)(q35;q14), which results in the formation of the fusion oncogenes PAX7-FOXO1 and PAX3-FOXO1, respectively. Since patients with fusion-positive ARMS (FP-RMS) have a poor prognosis and are treated with an aggressive therapeutic regimen, correct classification is of clinical importance. Detection of the translocation by different molecular methods is used f...
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#1Tamara KazimovaH-Index: 1
#2Fabienne TschanzH-Index: 1
Last. Martin PruschyH-Index: 28
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Placental growth factor (PlGF) is a pro-angiogenic, N-glycosylated growth factor, which is secreted under pathological situations. Here, we investigated the regulation of PlGF in response to ionizing radiation (IR) and its role for tumor angiogenesis and radiosensitivity. Secretion and expression of PlGF was induced in multiple tumor cell lines (medulloblastoma, colon and lung adenocarcinoma) in response to irradiation in a dose- and time-dependent manner. Early upregulation of PlGF expression a...
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#1Lisa BierbaumerH-Index: 2
#2Anna M. KatschnigH-Index: 5
Last. Raphaela SchwentnerH-Index: 15
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Ewing sarcoma (EwS) is a highly metastatic bone cancer characterized by the ETS fusion oncoprotein EWS-FLI1. EwS cells are phenotypically highly plastic and switch between functionally distinct cell states dependent on EWS-FLI1 fluctuations. Whereas EWS-FLI1high cells proliferate, EWS-FLI1low cells are migratory and invasive. Recently, we reported activation of MRTFB and TEAD, effectors of RhoA and Hippo signalling, upon low EWS-FLI1, orchestrating key steps of the EwS migratory gene expression ...
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#1Dominik LaubscherH-Index: 3
#2Berkley E. GryderH-Index: 18
Last. Javed KhanH-Index: 27
view all 20 authors...
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#1Joana G. Marques (Boston Children's Hospital)H-Index: 3
#2Berkley E. Gryder (NIH: National Institutes of Health)H-Index: 18
Last. Beat W. Schäfer (Boston Children's Hospital)H-Index: 71
view all 8 authors...
Cancer-specific chromosomal aberrations producing chimeric fusion genes are recurrently found in pediatric sarcomas. Fusion positive rhabdomyosarcoma (FP-RMS) and Ewing sarcoma (ES) are two rare but lethal pediatric malignancies driven by such chromosomal translocations. PAX3-FOXO1 and EWS-FLI1 are the most common products of the fusion genes found in FP-RMS and ES, respectively, and they are commonly perceived as the founding genetic abnormality driving the development of these malignancies by ...
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