Results 31 to 40 of about 178 (122)

Cell-based small-molecule compound screen identifies fenretinide as potential therapeutic for translocation-positive rhabdomyosarcoma. [PDF]

PLoS ONE, 2013
A subset of paediatric sarcomas are characterized by chromosomal translocations encoding specific oncogenic transcription factors. Such fusion proteins represent tumor specific therapeutic targets although so far it has not been possible to directly ...
David Herrero Martín, Aleksandar Boro, Beat W Schäfer   +2 more
doaj   +1 more source

PLK1 Phosphorylates PAX3-FOXO1, the Inhibition of Which Triggers Regression of Alveolar Rhabdomyosarcoma [PDF]

Cancer Research, 2015
Abstract Pediatric tumors harbor very low numbers of somatic mutations and therefore offer few targets to improve therapeutic management with targeted drugs. In particular, outcomes remain dismal for patients with metastatic alveolar rhabdomyosarcoma (aRMS), where the chimeric transcription factor PAX3/7-FOXO1 has been implicated but ...
Thalhammer Verena, Lopez-Garcia Laura A, Herrero Martin David, Hecker Regina, Laubscher Dominik, Gierisch Maria E, Wachtel Marco, Bode Peter, Nanni Paolo, Blank Bernd, Koscielniak Ewa, Schäfer Beat W   +11 more
openaire   +4 more sources

Evidence of pioneer factor activity of an oncogenic fusion transcription factor

iScience, 2021
Summary: Recent characterizations of pioneer transcription factors provide insights into their structures and patterns of chromatin recognition associated with their roles in cell fate commitment and transformation.
Benjamin D. Sunkel, Meng Wang, Stephanie LaHaye, Benjamin J. Kelly, James R. Fitch, Frederic G. Barr, Peter White, Benjamin Z. Stanton   +7 more
doaj   +1 more source

Rhabdomyosarcoma fusion oncoprotein initially pioneers a neural signature in vivo

Cell Reports
Summary: Fusion-positive rhabdomyosarcoma is an aggressive pediatric cancer molecularly characterized by arrested myogenesis. The defining genetic driver, PAX3::FOXO1, encodes a chimeric gain-of-function transcription factor.
Jack Kucinski, Alexi Tallan, Cenny Taslim, Andrew M. Vontell, Katherine M. Silvius, Meng Wang, Matthew V. Cannon, Benjamin Z. Stanton, Genevieve C. Kendall   +8 more
doaj   +1 more source

Epigenetic regulator BMI1 promotes alveolar rhabdomyosarcoma proliferation and constitutes a novel therapeutic target

Molecular Oncology, 2021
Rhabdomyosarcoma (RMS) is an aggressive pediatric soft tissue sarcoma. There are two main subtypes of RMS, alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma.
Cara E. Shields, Sindhu Potlapalli, Selma M. Cuya‐Smith, Sarah K. Chappell, Dongdong Chen, Daniel Martinez, Jennifer Pogoriler, Komal S. Rathi, Shiv A. Patel, Kristianne M. Oristian, Corinne M. Linardic, John M. Maris, Karmella A. Haynes, Robert W. Schnepp   +13 more
doaj   +1 more source

Regulatory landscape fusion in rhabdomyosarcoma through interactions between the PAX3 promoter and FOXO1 regulatory elements

Genome Biology, 2017
Background The organisation of vertebrate genomes into topologically associating domains (TADs) is believed to facilitate the regulation of the genes located within them.
Cristina Vicente-García, Barbara Villarejo-Balcells, Ibai Irastorza-Azcárate, Silvia Naranjo, Rafael D. Acemel, Juan J. Tena, Peter W. J. Rigby, Damien P. Devos, Jose L. Gómez-Skarmeta, Jaime J. Carvajal   +9 more
doaj   +1 more source

Natural Killer Cells in Paediatric Soft Tissue Sarcomas: A Systematic Review

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Paediatric soft tissue sarcomas (pSTS) are a rare and heterogeneous group of malignant tumours arising in tissues of mesenchymal origin. The role of natural killer (NK) cells in pSTS remains poorly understood, with evidence fragmented across small preclinical studies and early‐phase clinical trials.
Raya Dean, Lara V. Graham, Christina Putnam, Rosa Gomes Alves Martins, Daniel T. Harding, Tim J. Underwood, Zoë S. Walters, Matthew D. Blunt   +7 more
wiley   +1 more source

The PAX-FOXO1s trigger fast trans-differentiation of chick embryonic neural cells into alveolar rhabdomyosarcoma with tissue invasive properties limited by S phase entry inhibition.

PLoS Genetics, 2020
The chromosome translocations generating PAX3-FOXO1 and PAX7-FOXO1 chimeric proteins are the primary hallmarks of the paediatric fusion-positive alveolar subtype of Rhabdomyosarcoma (FP-RMS).
Gloria Gonzalez Curto, Audrey Der Vartanian, Youcef El-Mokhtar Frarma, Line Manceau, Lorenzo Baldi, Selene Prisco, Nabila Elarouci, Frédéric Causeret, Daniil Korenkov, Muriel Rigolet, Frédéric Aurade, Aurélien De Reynies, Vincent Contremoulins, Frédéric Relaix, Orestis Faklaris, James Briscoe, Pascale Gilardi-Hebenstreit, Vanessa Ribes   +17 more
doaj   +1 more source

Targeting transcription factors associated with hemoglobinopathies: Lessons from successful interventions and implications for cancer

Molecular Oncology, EarlyView.
This review summarizes the transcription factors, repressive chromatin‐modifying complexes, and epigenetic mechanisms that control fetal hemoglobin repression. Notably, many regulators of γ‐globin silencing also function in transcriptional and epigenetic networks that drive cancer, highlighting opportunities to translate advances in hemoglobinopathy ...
Meigen Yu, Puspa Das, Xing Zhang, Xiaodong Cheng   +3 more
wiley   +1 more source

FGF7–FGFR2 autocrine signaling increases growth and chemoresistance of fusion‐positive rhabdomyosarcomas

Molecular Oncology, 2022
Rhabdomyosarcomas are aggressive pediatric soft‐tissue sarcomas and include high‐risk PAX3–FOXO1 fusion‐gene‐positive cases. Fibroblast growth factor receptor 4 (FGFR4) is known to contribute to rhabdomyosarcoma progression; here, we sought to ...
Christopher I. Milton, Joanna Selfe, Ewa Aladowicz, Stella Y. K. Man, Carolina Bernauer, Edoardo Missiaglia, Zoë S. Walters, Susanne A. Gatz, Anna Kelsey, Melanie Generali, Gary Box, Melanie Valenti, Alexis deHaven‐Brandon, David Galiwango, Angela Hayes, Matthew Clarke, Elisa Izquierdo, David Gonzalez De Castro, Florence I. Raynaud, Vladimir Kirkin, Janet M. Shipley   +20 more
doaj   +1 more source