{"id":77889,"date":"2022-10-10T14:54:44","date_gmt":"2022-10-10T12:54:44","guid":{"rendered":"https:\/\/www.pcb.ub.edu\/desarrollan-un-modelo-de-estudio-del-sarcoma-de-ewing-en-la-mosca-drosophila\/"},"modified":"2022-10-11T17:14:55","modified_gmt":"2022-10-11T15:14:55","slug":"desarrollan-un-modelo-de-estudio-del-sarcoma-de-ewing-en-la-mosca-drosophila","status":"publish","type":"post","link":"https:\/\/www.pcb.ub.edu\/es\/desarrollan-un-modelo-de-estudio-del-sarcoma-de-ewing-en-la-mosca-drosophila\/","title":{"rendered":"Desarrollan un modelo de estudio del sarcoma de Ewing en Drosophila"},"content":{"rendered":"<p><strong>Investigadores liderados por el\u00a0Dr. Cayetano Gonz\u00e1lez, profesor de investigaci\u00f3n ICREA en el <a href=\"https:\/\/www.pcb.ub.edu\/es\/empresa\/institut-de-recerca-biomedica-irb-barcelona\/\" target=\"_blank\" rel=\"noopener\">IRB Barcelona<\/a>, con sede en el Parque Cient\u00edfico de Barcelona, y el Dr. Jaume Mora, director cient\u00edfico del <a href=\"https:\/\/www.sjdhospitalbarcelona.org\/es\/hospital\/proyectos-estrategicos\/pediatric-cancer-center-barcelona\" target=\"_blank\" rel=\"noopener\">SJD Pediatric Cancer Center Barcelona<\/a>, han dise\u00f1ado cepas transg\u00e9nicas de Drosophila que expresan una variante mutante del oncog\u00e9n humano denominada EWS-FLIFS. Se trata de un modelo animal in\u00e9dito que puede utilizarse para investigar el sarcoma de Ewing y cribar compuestos que puedan servir para el desarrollo de f\u00e1rmacos. El trabajo se publica en PNAS Nexus.<\/strong><\/p>\n<p>El sarcoma de Ewing es el segundo tumor \u00f3seo m\u00e1s frecuente en ni\u00f1os, adolescentes y adultos j\u00f3venes. No existe un tratamiento espec\u00edfico para esta enfermedad y el tratamiento actual sigue limit\u00e1ndose a la cirug\u00eda, la radioterapia y la quimioterapia. La supervivencia a largo plazo de los pacientes con sarcoma de Ewing metast\u00e1sico o recidivante es muy baja.<\/p>\n<p>El sarcoma de Ewing est\u00e1 causado por un \u00fanico oncog\u00e9n que resulta de la fusi\u00f3n de dos genes. Aunque pueden estar implicados varios genes, el EWSR1 y el FLI1 y el oncog\u00e9n resultante, conocido como EWS-FLI, son los responsables en la mayor\u00eda de los pacientes. A diferencia de la mayor\u00eda de los dem\u00e1s tipos de c\u00e1ncer, los intentos de desarrollar modelos animales experimentales de sarcoma de Ewing en ratones (que expresen el oncog\u00e9n EWS-FLI) han fracasado hasta la fecha.<\/p>\n<p>Impulsados por la necesidad de un modelo gen\u00e9ticamente trazable que pudiera utilizarse para estudiar la enfermedad, los investigadores liderados por el\u00a0<strong>Dr. Cayetano Gonz\u00e1lez<\/strong>, profesor de investigaci\u00f3n ICREA en el IRB Barcelona, y el\u00a0<strong>Dr. Jaume Mora<\/strong>, director cient\u00edfico del SJD Pediatric Cancer Center Barcelona (PCCB), han dise\u00f1ado cepas transg\u00e9nicas de\u00a0Drosophila\u00a0que expresan una variante mutante del oncog\u00e9n humano denominada EWS-FLIFS. Sorprendentemente, han descubierto que la expresi\u00f3n de la prote\u00edna humana EWS-FLIFS en ciertos tipos de c\u00e9lulas de\u00a0<em>Drosophila<\/em>\u00a0desencadena las mismas v\u00edas oncog\u00e9nicas que se sabe que explican la actividad oncog\u00e9nica de EWS-FLI en pacientes humanos.<\/p>\n<div id=\"attachment_77802\" style=\"width: 948px\" class=\"wp-caption alignnone\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-77802\" class=\"wp-image-77802 size-full\" src=\"https:\/\/www.pcb.ub.edu\/wp-content\/uploads\/2022\/10\/PNASNexus_Gonzalez_GraphicalAbstract.png.webp\" alt=\"\" width=\"938\" height=\"436\" srcset=\"https:\/\/www.pcb.ub.edu\/wp-content\/uploads\/2022\/10\/PNASNexus_Gonzalez_GraphicalAbstract.png.webp 938w, https:\/\/www.pcb.ub.edu\/wp-content\/uploads\/2022\/10\/PNASNexus_Gonzalez_GraphicalAbstract.png-300x139.webp 300w, https:\/\/www.pcb.ub.edu\/wp-content\/uploads\/2022\/10\/PNASNexus_Gonzalez_GraphicalAbstract.png-768x357.webp 768w\" sizes=\"auto, (max-width: 938px) 100vw, 938px\" \/><p id=\"caption-attachment-77802\" class=\"wp-caption-text\">Source: IRB Barcelona.<\/p><\/div>\n<h3><strong>Iluminaci\u00f3n de dos v\u00edas oncog\u00e9nicas<\/strong><\/h3>\n<p>Bas\u00e1ndose en su nueva l\u00ednea transg\u00e9nica de\u00a0<em>Drosophila<\/em>, los autores han \u201crecableado\u201d dos v\u00edas oncog\u00e9nicas utilizadas por EWS-FLI, de manera que cuando se activan por la presencia de EWS-FLIFS, dan lugar a la expresi\u00f3n de una prote\u00edna fluorescente que de otro modo nunca se expresar\u00eda. As\u00ed, en lugar del crecimiento del tumor, los investigadores utilizan la fluorescencia como lectura de la actividad oncog\u00e9nica de EWS-FLI.<\/p>\n<p>\u00abEste sencillo truco gen\u00e9tico facilita enormemente la realizaci\u00f3n de cribados gen\u00e9ticos y farmacol\u00f3gicos masivos para identificar \u00abmodificadores\u00bb que inhiban la actividad oncog\u00e9nica de EWS-FLI como inhibidores de la aparici\u00f3n de la fluorescencia\u00bb, explica la\u00a0<strong>Dra. Cristina Molnar<\/strong>, investigadora postdoctoral del IRB Barcelona y primera autora del estudio.<\/p>\n<p>Los cribados gen\u00e9ticos basados en este nuevo modelo permitir\u00e1n descubrir las prote\u00ednas cr\u00edticas necesarias para que EWS-FLI ejerza su funci\u00f3n oncog\u00e9nica, ampliando as\u00ed el conocimiento de las bases moleculares de la enfermedad, as\u00ed como identificar nuevas posibles dianas terap\u00e9uticas.\u00a0 Los cribados farmacol\u00f3gicos pueden identificar compuestos que podr\u00edan servir como mol\u00e9culas principales para el desarrollo de f\u00e1rmacos.<\/p>\n<p><strong>\u00bb M\u00e1s informaci\u00f3n: <a href=\"https:\/\/www.irbbarcelona.org\/es\/news\/cientificas\/cientificos-del-irb-barcelona-y-del-pccb-desarrollan-un-modelo-de-estudio-del\" target=\"_blank\" rel=\"noopener\">web del IRB Barcelona [+]<\/a><\/strong><\/p>\n<p><strong>\u00bb Art\u00edculo de referencia:<\/strong>\u00a0Cristina Molnar, Jose Reina, Anastasia Herrero, Jan Peter Heinen, Victoria M\u00e9ndiz, Sophie Bonnal, Manuel Irimia, Mar\u00eda S\u00e1nchez-Jim\u00e9nez, Sara S\u00e1nchez-Molina, Jaume Mora, Cayetano Gonzalez, Human EWS-FLI protein recapitulates in Drosophila the neomorphic functions that induce Ewing sarcoma tumorigenesis,\u00a0<em>PNAS Nexus<\/em>, 2022;, pgac222,\u00a0<a href=\"https:\/\/doi.org\/10.1093\/pnasnexus\/pgac222\" target=\"_blank\" rel=\"noopener\" data-google-interstitial=\"false\">https:\/\/doi.org\/10.1093\/pnasnexus\/pgac222<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Investigadores liderados por el\u00a0Dr. Cayetano Gonz\u00e1lez, profesor de investigaci\u00f3n ICREA en el IRB Barcelona, con sede en el Parque Cient\u00edfico de Barcelona, y el Dr. Jaume Mora, director cient\u00edfico del&#8230;<\/p>\n","protected":false},"author":14,"featured_media":77790,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"inline_featured_image":false,"footnotes":""},"categories":[24],"tags":[],"class_list":["post-77889","post","type-post","status-publish","format-standard","has-post-thumbnail","category-ciencia"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.0 - 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