
{"id":111403,"date":"2023-09-29T10:13:55","date_gmt":"2023-09-29T08:13:55","guid":{"rendered":"https:\/\/ibecbarcelona.eu\/an-artificial-muscle-to-study-duchenne-muscular-dystrophy\/"},"modified":"2025-03-05T10:01:22","modified_gmt":"2025-03-05T09:01:22","slug":"an-artificial-muscle-to-study-duchenne-muscular-dystrophy","status":"publish","type":"post","link":"https:\/\/ibecbarcelona.eu\/es\/un-musculo-artificial-para-estudiar-la-distrofia-muscular-de-duchenne","title":{"rendered":"Un m\u00fasculo artificial para estudiar la distrofia muscular de Duchenne"},"content":{"rendered":"\n<p><strong>El sistema, desarrollado por el IBEC, est\u00e1 fabricado a partir de c\u00e9lulas de pacientes y es el primer modelo 3D de m\u00fasculo capaz de reproducir el da\u00f1o que provoca la distrofia muscular de Duchenne.<\/strong> <strong>El siguiente paso ser\u00e1 fabricar una plataforma de \u00f3rgano-en-un-chip que permita llevar a cabo estudios precl\u00ednicos de f\u00e1rmacos contra la enfermedad y monitorizar el da\u00f1o muscular de manera m\u00e1s eficiente.<\/strong> <strong>El trabajo ha recibido financiaci\u00f3n de Duchenne Parent Project Espa\u00f1a, una asociaci\u00f3n sin \u00e1nimo de lucro dirigida por las familias de ni\u00f1os afectados por este tipo de distrofia.<\/strong><\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" width=\"2560\" height=\"1440\" data-src=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/Duchenne-JFernandez-ATejedera-edited-scaled.jpg\" alt=\"\" class=\"wp-image-111359 lazyload\" data-srcset=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/Duchenne-JFernandez-ATejedera-edited-scaled.jpg 2560w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/Duchenne-JFernandez-ATejedera-edited-300x169.jpg 300w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/Duchenne-JFernandez-ATejedera-edited-1024x576.jpg 1024w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/Duchenne-JFernandez-ATejedera-edited-768x432.jpg 768w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/Duchenne-JFernandez-ATejedera-edited-1536x864.jpg 1536w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/Duchenne-JFernandez-ATejedera-edited-2048x1152.jpg 2048w\" data-sizes=\"(max-width: 2560px) 100vw, 2560px\" src=\"data:image\/svg+xml;base64,PHN2ZyB3aWR0aD0iMSIgaGVpZ2h0PSIxIiB4bWxucz0iaHR0cDovL3d3dy53My5vcmcvMjAwMC9zdmciPjwvc3ZnPg==\" style=\"--smush-placeholder-width: 2560px; --smush-placeholder-aspect-ratio: 2560\/1440;\" \/><figcaption class=\"wp-element-caption\">Juanma Fern\u00e1ndez y Ainoa Tejedera, investigadores del Instituto de Bioingenier\u00eda de Catalu\u00f1a (IBEC).<\/figcaption><\/figure>\n\n\n\n<p>La Distrofia Muscular de Duchenne (DMD) es la distrofia muscular m\u00e1s com\u00fan diagnosticada durante la infancia, con alrededor de 20.000 casos nuevos cada a\u00f1o. Se trata de un desorden progresivo del m\u00fasculo que causa la p\u00e9rdida de su funci\u00f3n de manera que los afectados terminan perdiendo totalmente su independencia y sufriendo problemas m\u00e9dicos graves, con una expectativa de vida promedio de 30 a\u00f1os.<\/p>\n\n\n\n<p>La causa de la enfermedad es una mutaci\u00f3n en el gen que codifica la distrofina, una prote\u00edna que amortigua el impacto de la contracci\u00f3n de los m\u00fasculos en la membrana de las c\u00e9lulas. Debido a que la distrofina est\u00e1 ausente, las c\u00e9lulas musculares se da\u00f1an f\u00e1cilmente.<\/p>\n\n\n\n<p>En la actualidad, no existe una cura para la DMD y uno de los desaf\u00edos principales con los que se encuentra la comunidad investigadora es crear modelos artificiales que consigan recrear de manera eficaz el da\u00f1o presente en los m\u00fasculos de los pacientes, para poder estudiar nuevos tratamientos en el laboratorio.<\/p>\n\n\n\n<p>Un estudio del Instituto de Bioingenier\u00eda de Catalu\u00f1a (IBEC) publicado esta semana en la revista <em>Biofabrication,<\/em> describe el desarrollo de un modelo 3D de m\u00fasculo que es capaz de reproducir el da\u00f1o presente en el tejido muscular de las personas afectadas por la distrofia muscular de Duchenne.<\/p>\n\n\n\n<p>El sistema, fabricado a partir de ingenier\u00eda de tejidos con c\u00e9lulas de pacientes, contiene fibras musculares capaces de contraerse al aplicar un est\u00edmulo el\u00e9ctrico. Una caracter\u00edstica esencial para crear un modelo artificial de m\u00fasculo que permita llevar a cabo estudios precl\u00ednicos de f\u00e1rmacos para tratar la DMD.<\/p>\n\n\n\n<p>El trabajo lo ha liderado Juanma Fern\u00e1ndez Costa, investigador senior del IBEC, con Ainoa Tejedera Villafranca, estudiante de doctorado del IBEC, como primera autora. Ambos pertenecen al grupo de <a href=\"https:\/\/ibecbarcelona.eu\/es\/research-groups\/biosensors-for-bioengineering\/\">Biosensores para bioingenier\u00eda<\/a>, liderado por el profesor de investigaci\u00f3n ICREA Javier Ram\u00f3n Azc\u00f3n.<\/p>\n\n\n\n<blockquote class=\"wp-block-quote is-layout-flow wp-block-quote-is-layout-flow\">\n<p>\u201cLo novedoso de este estudio es que hemos buscado modelizar la causa principal de la enfermedad, que es el da\u00f1o del sarcolema, la membrana de las c\u00e9lulas musculares. Para nosotros era muy importante poder replicarlo en el laboratorio y lo hemos conseguido. No se hab\u00eda hecho antes\u201d, comenta Juanma Fern\u00e1ndez.<\/p>\n<\/blockquote>\n\n\n\n<p><strong>\u201cTrabajamos mucho tiempo en diferentes protocolos hasta conseguir que apareciera ese da\u00f1o en las c\u00e9lulas de pacientes, pero no en las de control, de personas sin Duchenne. Es delicado, porque si estimulas el m\u00fasculo, puedes causar rotura de fibras tambi\u00e9n en las c\u00e9lulas sanas, igual que ocurre cuando hacemos deporte y tenemos agujetas\u201d<\/strong>, a\u00f1ade Ainoa Tejedera.<\/p>\n\n\n\n<p>El objetivo de los investigadores era conseguir un modelo que permitiera comprobar si los f\u00e1rmacos son capaces de revertir ese da\u00f1o en las c\u00e9lulas. No atacar los s\u00edntomas, que es en lo que se centran los tratamientos paliativos, sino ir al origen de la enfermedad.<\/p>\n\n\n\n<p>Aunque ya han testado algunos f\u00e1rmacos en este modelo, est\u00e1n trabajando en desarrollar un modelo mejorado, conocido como \u00f3rgano-en-un-chip. Se trata de una plataforma m\u00e1s avanzada que a\u00f1ade sensores y un sistema de microflu\u00eddica al modelo de m\u00fasculo 3D. Esto permitir\u00e1 monitorizar el da\u00f1o celular de forma m\u00e1s eficiente y testar diferentes mol\u00e9culas o f\u00e1rmacos con mayor rapidez.<\/p>\n\n\n\n<div style=\"height:20px\" aria-hidden=\"true\" class=\"wp-block-spacer\"><\/div>\n\n\n\n<div class=\"wp-block-columns is-layout-flex wp-container-core-columns-is-layout-9d6595d7 wp-block-columns-is-layout-flex\">\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\" style=\"flex-basis:66.66%\">\n<figure class=\"wp-block-video\"><video controls src=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/bfacfb3dsupp2.m4v\"><\/video><figcaption class=\"wp-element-caption\">Video of 3D skeletal muscle tissue<br>responding to electrical pulse stimulation.<\/figcaption><\/figure>\n<\/div>\n\n\n\n<div class=\"wp-block-column is-layout-flow wp-block-column-is-layout-flow\" style=\"flex-basis:33.33%\">\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" width=\"1024\" height=\"768\" data-src=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/processed-ABE7A3DD-038B-4911-94A0-EDEE54A9A988-3CB0CFD8-5E87-40B5-8788-9C5BD407A386-1024x768.jpeg\" alt=\"\" class=\"wp-image-111364 lazyload\" data-srcset=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/processed-ABE7A3DD-038B-4911-94A0-EDEE54A9A988-3CB0CFD8-5E87-40B5-8788-9C5BD407A386-1024x768.jpeg 1024w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/processed-ABE7A3DD-038B-4911-94A0-EDEE54A9A988-3CB0CFD8-5E87-40B5-8788-9C5BD407A386-300x225.jpeg 300w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/processed-ABE7A3DD-038B-4911-94A0-EDEE54A9A988-3CB0CFD8-5E87-40B5-8788-9C5BD407A386-768x576.jpeg 768w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/processed-ABE7A3DD-038B-4911-94A0-EDEE54A9A988-3CB0CFD8-5E87-40B5-8788-9C5BD407A386-1536x1153.jpeg 1536w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2023\/09\/processed-ABE7A3DD-038B-4911-94A0-EDEE54A9A988-3CB0CFD8-5E87-40B5-8788-9C5BD407A386-2048x1537.jpeg 2048w\" data-sizes=\"(max-width: 1024px) 100vw, 1024px\" src=\"data:image\/svg+xml;base64,PHN2ZyB3aWR0aD0iMSIgaGVpZ2h0PSIxIiB4bWxucz0iaHR0cDovL3d3dy53My5vcmcvMjAwMC9zdmciPjwvc3ZnPg==\" style=\"--smush-placeholder-width: 1024px; --smush-placeholder-aspect-ratio: 1024\/768;\" \/><figcaption class=\"wp-element-caption\">Image of the 3D muscle model.<\/figcaption><\/figure>\n<\/div>\n<\/div>\n\n\n\n<h4 class=\"wp-block-heading\"><strong>La importancia de colaborar con los pacientes y sus familias<\/strong><\/h4>\n\n\n\n<p>El trabajo, que forma parte de la tesis doctoral de Tejedera, ha recibido financiaci\u00f3n de Duchenne Parent Project Espa\u00f1a (DPPE), una asociaci\u00f3n sin \u00e1nimo de lucro creada y dirigida por madres y padres de ni\u00f1os con Distrofia Muscular de Duchenne y Becker.<\/p>\n\n\n\n<p>Esta colaboraci\u00f3n representa otra manera de hacer llegar la ciencia a la sociedad. En palabras de Fern\u00e1ndez: <strong>\u201ces un ejemplo fant\u00e1stico de c\u00f3mo los investigadores podemos hacer pi\u00f1a con los pacientes y sus familias para buscar objetivos comunes\u201d.<\/strong><\/p>\n\n\n\n<p>Silvia \u00c1vila, presidenta de la asociaci\u00f3n, destaca que <strong>\u201cEs un orgullo saber que hemos financiado un proyecto que puede acelerar el proceso de investigaci\u00f3n, a trav\u00e9s de la creaci\u00f3n de un m\u00fasculo artificial en un chip. Por un lado, reducimos el uso de animales en laboratorio y, por otro lado, acortamos el tiempo de convertir una posible terapia en realidad. B\u00e1sico para esta enfermedad que va restando tiempo y calidad de vida a nuestros hijos\u00bb<\/strong>.<\/p>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<p><strong>Referenced article:<\/strong><\/p>\n\n\n\n<p>Ainoa Tejedera-Villafranca, Marisol Montolio, Javier Ram\u00f3n-Azc\u00f3n, and Juan M Fern\u00e1ndez-Costa. <a href=\"https:\/\/iopscience.iop.org\/article\/10.1088\/1758-5090\/acfb3d\">Mimicking sarcolemmal damage in vitro: a contractile 3D model of skeletal muscle for drug testing in Duchenne muscular dystrophy<\/a>. <em>Biofabrication<\/em> (2023). DOI: 10.1088\/1758-5090\/acfb3d<\/p>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<p><em>Ayuda financiada con el apoyo del programa predoctoral AGAUR-FI ayduas (2023 FI-2 00386) Joan Or\u00f3 de la Generalitat de Catalunya y del Fons Europeu Social Plus  <\/em><\/p>\n\n\n\n<figure class=\"wp-block-image size-full\"><img decoding=\"async\" width=\"891\" height=\"116\" data-src=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2022\/11\/logosfse-1.jpg\" alt=\"\" class=\"wp-image-124540 lazyload\" style=\"--smush-placeholder-width: 891px; --smush-placeholder-aspect-ratio: 891\/116;object-fit:cover\" data-srcset=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2022\/11\/logosfse-1.jpg 891w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2022\/11\/logosfse-1-300x39.jpg 300w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2022\/11\/logosfse-1-768x100.jpg 768w\" data-sizes=\"(max-width: 891px) 100vw, 891px\" src=\"data:image\/svg+xml;base64,PHN2ZyB3aWR0aD0iMSIgaGVpZ2h0PSIxIiB4bWxucz0iaHR0cDovL3d3dy53My5vcmcvMjAwMC9zdmciPjwvc3ZnPg==\" \/><\/figure>\n\n\n\n<p><\/p>\n","protected":false},"excerpt":{"rendered":"<p>El sistema, desarrollado por el IBEC, est\u00e1 fabricado a partir de c\u00e9lulas de pacientes y es el primer modelo 3D de m\u00fasculo capaz de reproducir el da\u00f1o que provoca la distrofia muscular de Duchenne. El siguiente paso ser\u00e1 fabricar una plataforma de \u00f3rgano-en-un-chip que permita llevar a cabo estudios precl\u00ednicos de f\u00e1rmacos contra la enfermedad y monitorizar el da\u00f1o muscular de manera m\u00e1s eficiente. El trabajo ha recibido financiaci\u00f3n de Duchenne Parent Project Espa\u00f1a, una asociaci\u00f3n sin \u00e1nimo de lucro dirigida por las familias de ni\u00f1os afectados por este tipo de distrofia.<\/p>\n","protected":false},"author":37,"featured_media":111358,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[97],"tags":[2001,1851,1130],"post_formats":[],"class_list":["post-111403","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-noticias-de-investigacion","tag-biosensors-for-bioengineering-es","tag-enfermedades-neuromusculares","tag-javier-ramon-es"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.3 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Un m\u00fasculo artificial para estudiar la distrofia muscular de Duchenne - Institute for Bioengineering of Catalonia<\/title>\n<meta name=\"description\" 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