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oai:recercat.cat:2445/1787052026-02-05T03:21:22Zcom_2072_1057col_2072_478798col_2072_478799col_2072_478916col_2072_478917

00925njm 22002777a 4500 dc Huertas-Martínez, Juan author Rello Varona, Santiago author Herrero Martín, David author Barrau, Ignasi author Garcia Monclús, Silvia author Sáinz Jaspeado, Miguel Guillermo author Lagares Tena, Laura author Núñez Álvarez, Yaiza author Mateo Lozano, Silvia author Mora Graupera, Jaume author Roma, Josep author Toran, Nuria author Moran, Sebastian author López Alemany, Roser author Gallego, Soledad author Esteller, Manel, 1968- author Peinado Morales, Miguel Á. (Miguel Ángel) author García del Muro Solans, Xavier author Tirado, Oscar M. author 2021-06-29T16:04:39Z 2021-06-29T16:04:39Z 2014-10-30 2021-06-29T16:04:39Z Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence. Despite advances in therapy, patients with histological variant of rhabdomyosarcoma known as alveolar rhabdomyosarcoma (ARMS) have a 5-year survival of less than 30%. Caveolin-1 (CAV1), encoding the structural component of cellular caveolae, is a suggested tumor suppressor gene involved in cell signaling. In the present study we report that compared to other forms of rhabdomyosarcoma (RMS) CAV1 expression is either undetectable or very low in ARMS cell lines and tumor samples. DNA methylation analysis of the promoter region and azacytidine-induced re-expression suggest the involvement of epigenetic mechanisms in the silencing of CAV1. Reintroduction of CAV1 in three of these cell lines impairs their clonogenic capacity and promotes features of muscular differentiation. In vitro, CAV1-expressing cells show high expression of Caveolin-3 (CAV3), a muscular differentiation marker. Blockade of MAPK signaling is also observed. In vivo, CAV1-expressing xenografts show growth delay, features of muscular differentiation and increased cell death. In summary, our results suggest that CAV1 could function as a potent tumor suppressor in ARMS tumors. Inhibition of CAV1 function therefore, could contribute to aberrant cell proliferation, leading to ARMS development. Metabolisme Patologia Tumors Metabolism Pathology Tumors Caveolin-1 is down-regulated in alveolar habdomyosarcomas and negatively regulates tumor growth