Sequential combinations of chemotherapeutic agents with BH3 mimetics to treat rhabdomyosarcoma and avoid resistance

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood and adolescence. Refractory/relapsed RMS patients present a bad prognosis that combined with the lack of specific biomarkers impairs the development of new therapies. Here, we utilize dynamic BH3 profiling (DBP), a functional...

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Detalles Bibliográficos
Autores: Alcon, Clara, Manzano-Muñoz, Albert|||0000-0003-4039-7391, Prada, Estela|||0000-0003-4611-1120, Català-Mora, Jaume|||0000-0003-2152-8579, Soriano, Aroa|||0000-0001-9659-1471, Guillén, Gabriela|||0000-0001-5632-2672, Gallego, Soledad|||0000-0002-4712-9624, Roma, Josep|||0000-0001-7692-6123, Samitier, Josep|||0000-0002-1140-3679, Villanueva, Alejandro|||0000-0001-5164-0006, Montero, Joan|||0000-0002-9192-4836
Tipo de recurso: artículo
Fecha de publicación:2020
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:252849
Acceso en línea:https://ddd.uab.cat/record/252849
https://dx.doi.org/urn:doi:10.1038/s41419-020-02887-y
Access Level:acceso abierto
Palabra clave:Paediatric cancer
Predictive markers
Descripción
Sumario:Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood and adolescence. Refractory/relapsed RMS patients present a bad prognosis that combined with the lack of specific biomarkers impairs the development of new therapies. Here, we utilize dynamic BH3 profiling (DBP), a functional predictive biomarker that measures net changes in mitochondrial apoptotic signaling, to identify anti-apoptotic adaptations upon treatment. We employ this information to guide the use of BH3 mimetics to specifically inhibit BCL-2 pro-survival proteins, defeat resistance and avoid relapse. Indeed, we found that BH3 mimetics that selectively target anti-apoptotic BCL-xL and MCL-1, synergistically enhance the effect of clinically used chemotherapeutic agents vincristine and doxorubicin in RMS cells. We validated this strategy in vivo using a RMS patient-derived xenograft model and observed a reduction in tumor growth with a tendency to stabilization with the sequential combination of vincristine and the MCL-1 inhibitor S63845. We identified the molecular mechanism by which RMS cells acquire resistance to vincristine: an enhanced binding of BID and BAK to MCL-1 after drug exposure, which is suppressed by subsequently adding S63845. Our findings validate the use of DBP as a functional assay to predict treatment effectiveness in RMS and provide a rationale for combining BH3 mimetics with chemotherapeutic agents to avoid tumor resistance, improve treatment efficiency, and decrease undesired secondary effects.