{"id":111402,"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":"2025-03-05T10:00:32","modified_gmt":"2025-03-05T09:00:32","slug":"an-artificial-muscle-to-study-duchenne-muscular-dystrophy","status":"publish","type":"post","link":"https:\/\/ibecbarcelona.eu\/ca\/un-muscul-artificial-per-estudiar-la-distrofia-muscular-de-duchenne","title":{"rendered":"Un m\u00fascul artificial per estudiar la distr\u00f2fia muscular de Duchenne"},"content":{"rendered":"\n

El sistema, desenvolupat per l’IBEC, est\u00e0 fabricat a partir de c\u00e8l\u00b7lules de pacients i \u00e9s el primer model 3D de m\u00fascul capa\u00e7 de reproduir el dany que provoca la distr\u00f2fia muscular de Duchenne.<\/strong> El seg\u00fcent pas ser\u00e0 fabricar una plataforma d’ \u00f2rgan-en-un-xip que permeti dur a terme estudis precl\u00ednics de f\u00e0rmacs contra la malaltia i monitorar el dany muscular de manera m\u00e9s eficient.<\/strong> El treball ha rebut finan\u00e7ament de Duchenne Parent Project Espanya, una associaci\u00f3 sense \u00e0nim de lucre dirigida per les fam\u00edlies de nens afectats per aquest tipus de distr\u00f2fia.<\/strong><\/p>\n\n\n\n

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Juanma Fern\u00e1ndez i Ainoa Tejedera, investigadors de l’Institut de Bioenginyeria de Catalunya (IBEC).<\/figcaption><\/figure>\n\n\n\n

La Distr\u00f2fia Muscular de Duchenne (DMD) \u00e9s la distr\u00f2fia muscular m\u00e9s comuna diagnosticada durant la inf\u00e0ncia, amb al voltant de 20.000 casos nous cada any. Es tracta d’un desordre progressiu del m\u00fascul que causa la p\u00e8rdua de la seva funci\u00f3 de manera que els afectats acaben perdent totalment la seva independ\u00e8ncia i patint problemes m\u00e8dics greus, amb una expectativa de vida mitjana de 30 anys.<\/p>\n\n\n\n

La causa de la malaltia \u00e9s una mutaci\u00f3 en el gen que codifica la distrofina, una prote\u00efna que esmorteeix l’impacte de la contracci\u00f3 dels m\u00fasculs en la membrana de les c\u00e8l\u00b7lules. At\u00e8s que la distrofina est\u00e0 absent, les c\u00e8l\u00b7lules musculars es malmeten f\u00e0cilment.<\/p>\n\n\n\n

En l’actualitat, no existeix una cura per a la DMD i un dels desafiaments principals amb els quals es troba la comunitat investigadora \u00e9s crear models artificials que aconsegueixin recrear de manera efica\u00e7 el dany present en els m\u00fasculs dels pacients, per poder estudiar nous tractaments al laboratori.<\/p>\n\n\n\n

Un estudi de l’IBEC publicat aquesta setmana a la revista Biofabrication,<\/em> descriu el desenvolupament d’un model 3D de m\u00fascul que \u00e9s capa\u00e7 de reproduir el dany present en el teixit muscular de les persones afectades per la distr\u00f2fia muscular de Duchenne.<\/p>\n\n\n\n

El sistema, fabricat a partir d’enginyeria de teixits amb c\u00e8l\u00b7lules de pacients, cont\u00e9 fibres musculars capaces de contreure’s en aplicar un est\u00edmul el\u00e8ctric. Una caracter\u00edstica essencial per crear un model artificial de m\u00fascul que permeti dur a terme estudis precl\u00ednics de f\u00e0rmacs per tractar la DMD.<\/p>\n\n\n\n

El treball l’ha liderat Juanma Fern\u00e1ndez Costa, investigador s\u00e8nior de l’IBEC, amb Ainoa Tejedera Villafranca, estudiant de doctorat de l’IBEC, com a primera autora. Tots dos pertanyen al grup de Biosensors per a bioenginyeria<\/a>, liderat pel professor d’investigaci\u00f3 ICREA Javier Ram\u00f3n Azc\u00f3n.<\/p>\n\n\n\n

\n

“L\u2019innovador d’aquest estudi \u00e9s que hem buscat modelitzar la causa principal de la malaltia, que \u00e9s el dany del sarcolemma, la membrana de les c\u00e8l\u00b7lules musculars. Per a nosaltres era molt important poder replicar-ho al laboratori i ho hem aconseguit. No s’havia fet abans”, comenta Juanma Fern\u00e1ndez.<\/p>\n<\/blockquote>\n\n\n\n

“Vam treballar molt de temps en diferents protocols fins a aconseguir que aparegu\u00e9s aquest dany en les c\u00e8l\u00b7lules de pacients, per\u00f2 no en les de control, de persones sense Duchenne. \u00c9s delicat, perqu\u00e8 si estimules el m\u00fascul, pots causar trencament de fibres tamb\u00e9 a les c\u00e8l\u00b7lules sanes, igual que passa quan sentim punxades despr\u00e9s de fer esport”<\/strong>, afegeix Ainoa Tejedera.<\/p>\n\n\n\n

L’objectiu dels investigadors era aconseguir un model que permet\u00e9s comprovar si els f\u00e0rmacs s\u00f3n capa\u00e7os de revertir aquest dany en les c\u00e8l\u00b7lules. No atacar els s\u00edmptomes, que \u00e9s en el que se centren els tractaments pal\u00b7liatius, sin\u00f3 anar a l’origen de la malaltia.<\/p>\n\n\n\n

Tot i que ja han testat alguns f\u00e0rmacs en aquest model, estan treballant a desenvolupar un model millorat, conegut com a \u00f2rgan-en-un-xip. Es tracta d’una plataforma m\u00e9s avan\u00e7ada que afegeix sensors i un sistema de microfluidica al model de m\u00fascul 3D. Aix\u00f2 permetr\u00e0 monitorar el dany cel\u00b7lular de forma m\u00e9s eficient i testar diferents mol\u00e8cules o f\u00e0rmacs amb m\u00e9s rapidesa.<\/p>\n\n\n\n

<\/div>\n\n\n\n
\n
\n
V\u00eddeo de teixit muscular esquel\u00e8tic 3D que respon a l’estimulaci\u00f3 de polsos el\u00e8ctrics.<\/figcaption><\/figure>\n<\/div>\n\n\n\n
\n
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Imatge del model de m\u00fascul 3D.<\/figcaption><\/figure>\n<\/div>\n<\/div>\n\n\n\n

La import\u00e0ncia de col\u00b7laborar amb els pacients i les seves fam\u00edlies<\/strong><\/h4>\n\n\n\n

El treball, que forma part de la tesi doctoral de Tejedera, ha rebut finan\u00e7ament de Duchenne Parent Project Espa\u00f1a (DPPE), una associaci\u00f3 sense \u00e0nim de lucre creada i dirigida per mares i pares de nens amb Distr\u00f2fia Muscular de Duchenne i Becker.<\/p>\n\n\n\n

Aquesta col\u00b7laboraci\u00f3 representa una altra manera de fer arribar la ci\u00e8ncia a la societat. En paraules de Fern\u00e1ndez: “\u00e9s un exemple fant\u00e0stic de com els investigadors podem fer pinya amb els pacients i les seves fam\u00edlies per buscar objectius comuns”.<\/strong><\/p>\n\n\n\n

Silvia \u00c1vila, presidenta de l’associaci\u00f3, destaca que “\u00c9s un orgull saber que hem finan\u00e7at un projecte que pot accelerar el proc\u00e9s d’investigaci\u00f3, a trav\u00e9s de la creaci\u00f3 d’un m\u00fascul artificial en un xip. D’una banda, redu\u00efm l’\u00fas d’animals en laboratori i, d’altra banda, escurcem el temps de convertir una possible ter\u00e0pia en realitat. B\u00e0sic per a aquesta malaltia que va restant temps i qualitat de vida als nostres fills”<\/strong>.<\/p>\n\n\n\n

\n\n\n\n

Article referenciat:<\/strong><\/p>\n\n\n\n

Ainoa Tejedera-Villafranca, Marisol Montolio, Javier Ram\u00f3n-Azc\u00f3n, and Juan M Fern\u00e1ndez-Costa. Mimicking sarcolemmal damage in vitro: a contractile 3D model of skeletal muscle for drug testing in Duchenne muscular dystrophy<\/a>. Biofabrication<\/em> (2023). DOI: 10.1088\/1758-5090\/acfb3d<\/p>\n\n\n\n

\n\n\n\n

Ajut finan\u00e7at amb el suport del programa predoctoral AGAUR-FI ajuts (2023 FI-2 00386) Joan Or\u00f3 de la Generalitat de Catalunya i del Fons Europeu Social Plus <\/em><\/p>\n\n\n\n

\"\"<\/figure>\n\n\n\n

<\/p>\n","protected":false},"excerpt":{"rendered":"

El sistema, desenvolupat per l’IBEC, est\u00e0 fabricat a partir de c\u00e8l\u00b7lules de pacients i \u00e9s el primer model 3D de m\u00fascul capa\u00e7 de reproduir el dany que provoca la distr\u00f2fia muscular de Duchenne. El seg\u00fcent pas ser\u00e0 fabricar una plataforma d’ \u00f2rgan-en-un-xip que permeti dur a terme estudis precl\u00ednics de f\u00e0rmacs contra la malaltia i monitorar el dany muscular de manera m\u00e9s eficient. El treball ha rebut finan\u00e7ament de Duchenne Parent Project Espanya, una associaci\u00f3 sense \u00e0nim de lucre dirigida per les fam\u00edlies de nens afectats per aquest tipus de distr\u00f2fia.<\/p>\n","protected":false},"author":37,"featured_media":111357,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[96],"tags":[1999,1131,1852],"post_formats":[],"class_list":["post-111402","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-noticies-de-recerca","tag-biosensors-for-bioengineering-ca","tag-javier-ramon-ca","tag-malalties-neuromusculars"],"yoast_head":"\nUn m\u00fascul artificial per estudiar la distr\u00f2fia muscular de Duchenne - Institute for Bioengineering of Catalonia<\/title>\n<meta name=\"description\" content=\"Fet a partir de c\u00e8l\u00b7lules de pacients, \u00e9s el primer model 3D de m\u00fascul capa\u00e7 de reproduir el dany que 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