{"id":29013,"date":"2017-05-05T10:14:45","date_gmt":"2017-05-05T08:14:45","guid":{"rendered":"http:\/\/ibecbarcelona.eu\/ca\/?p=29013"},"modified":"2017-05-15T11:51:02","modified_gmt":"2017-05-15T09:51:02","slug":"un-model-cel%c2%b7lular-per-ajudar-a-estudiar-la-relacio-entre-malalties-neurodegeneratives","status":"publish","type":"post","link":"https:\/\/ibecbarcelona.eu\/ca\/un-model-cel%c2%b7lular-per-ajudar-a-estudiar-la-relacio-entre-malalties-neurodegeneratives\/","title":{"rendered":"Un model cel\u00b7lular per ajudar a estudiar la relaci\u00f3 entre malalties neurodegeneratives"},"content":{"rendered":"<p>La capacitat de desenvolupar models cel\u00b7lulars amb caracter\u00edstiques de dues malalties neurodegeneratives no relacionades en les mateixes neurones podria ajudar en l&#8217;estudi d&#8217;altres dol\u00e8ncies neurodegeneratives amb diagn\u00f2stic similar, com l&#8217;Alzheimer.<\/p>\n<p>Aquest treball, publicat en la revista <em>Molecular Neurobiology<\/em>, \u00e9s el resultat de la col\u00b7laboraci\u00f3 entre experts del Grup de Neurobiologia Molecular i Cel\u00b7lular de l\u2019IBEC, i el neuropat\u00f2leg Isidre Ferrer, catedr\u00e0tic de la <a href=\"http:\/\/www.ub.edu\/medicina\/\" target=\"_self\">Facultat de Medicina i Ci\u00e8ncies de la Salut<\/a> i de l\u2019<a href=\"http:\/\/www.neurociencies.ub.edu\/recerca\/neurobiologia-i-neurofarmacologia\/jose-antonio-del-rio-fernandez\/\" target=\"_self\">Institut de Neuroci\u00e8ncies<\/a>, i el neur\u00f2leg Adolfo L\u00f3pez, de l\u2019Hospital Universitari Donostia de Sant Sebasti\u00e0. Va comen\u00e7ar quan els metges van analitzar el cervell d&#8217;un pacient mort i van revelar que, a m\u00e9s d&#8217;una tauopatia &#8211; un trastorn neurodegeneratiu -, el pacient tamb\u00e9 tenia una mutaci\u00f3 gen\u00e8tica que causa una altra malaltia neurodegenerativa molt m\u00e9s rara coneguda com a s\u00edndrome de Gerstmann-Str\u00e4ussler-Scheinker (GSS), una prionopatia que implica una mutaci\u00f3 del gen de la prote\u00efna pri\u00f2nica PRNP.<\/p>\n<p>Trobar dues malalties neurodegeneratives de diferent origen en el mateix pacient va encuriosir els metges, que van decidir contactar amb els investigadors de l\u2019IBEC per arribar al fons del misteri. Excepcionalment, els col\u00b7laboradors van comptar amb el gener\u00f3s suport d&#8217;un membre de la fam\u00edlia del pacient portador de la mateixa mutaci\u00f3, que va donar c\u00e8l\u00b7lules de la pell. Els investigadors van ser capa\u00e7os de reprogramar aquestes c\u00e8l\u00b7lules som\u00e0tiques i convertir-les en neurones per estudiar l&#8217;aparent relaci\u00f3 entre la tauopatia i la mutaci\u00f3 observada en el gen PRNP. &#8220;Les neurones generades en aquest estudi s\u00f3n la primera l\u00ednia de c\u00e8l\u00b7lules indu\u00efdes a partir de fibroblastos d&#8217;un pacient amb GSS&#8221;, diu Jos\u00e9 Antonio Del R\u00edo, professor de la Universitat de Barcelona i investigador principal a l \u2019IBEC, qui va coordinar la recerca.<\/p>\n<p>La reprogramaci\u00f3 de fibroblastos per generar neurones es va dur a terme en dues etapes. Durant la primera, els fibroblastos van ser reprogramats en c\u00e8l\u00b7lules mare pluripotents indu\u00efdes (c\u00e8l\u00b7lules iPS, de l&#8217;angl\u00e8s <em>induced pluripotent stem<\/em> cells). Aquesta transici\u00f3 va ser desenvolupada pel grup de l\u2019\u00c1ngel Raya, Director del Centre de Medicina Regenerativa de Barcelona (CMR[B]). El segon pas, que consisteix a diferenciar les c\u00e8l\u00b7lules iPS en neurones, es va dur a terme en els grups de Rosario S\u00e1nchez-Pernaute (Inbiomed), Joaqu\u00edn Castilla (CiCBiogune) i del Prof. Del R\u00edo de l\u2019IBEC. &#8220;Vam treballar en paral\u00b7lel a causa de la complexitat de crear neurones a partir de c\u00e8l\u00b7lules pluripotents. Afortunadament, vam trobar que els dos m\u00e8todes proposats generen amb \u00e8xit neurones completament funcionals&#8221;, diu Del R\u00edo.<\/p>\n<p>Les neurones diferenciades amb la mutaci\u00f3 PRNP tenien la seva funci\u00f3 natural alterada, donant lloc a un augment de la fosforilaci\u00f3 de tau &#8211; signe distintiu de la tauopatia -, desorganitzaci\u00f3 microtubular i mort cel\u00b7lular. Aquesta relaci\u00f3 funcional entre la PRNP i la degeneraci\u00f3 tau demostrada aqu\u00ed va ser observada pr\u00e8viament durant l&#8217;evoluci\u00f3 dels pacients amb malaltia d&#8217;Alzheimer en una publicaci\u00f3 dirigida per Rosalina Gav\u00edn, investigadora s\u00e8nior del laboratori del Prof. Del R\u00edo.<\/p>\n<p>La combinaci\u00f3 d&#8217;aquests dos processos en una \u00fanica l\u00ednia cel\u00b7lular obre la via per estudiar la seva possible relaci\u00f3 o co-ocurr\u00e8ncia en altres malalties neurodegeneratives com el Parkinson o l&#8217;Alzheimer, que en alguns pacients es desenvolupen juntament amb altres trastorns que impliquen mutacions en el gen PRNP. &#8220;Aquesta inesperada relaci\u00f3 obre la porta a la cerca de noves dianes terap\u00e8utiques que evitin que ambdues malalties es complementin per provocar la degeneraci\u00f3 i la mort neuronal&#8221;, afirma Andreu Matamoros, investigador del grup del Prof. Del R\u00edo a l\u2019IBEC i primer autor de l&#8217;estudi.<\/p>\n<p>El grup de l\u2019IBEC coordina la <em>Red Espa\u00f1ola de Excelencia en Investigaci\u00f3n Pri\u00f3nica<\/em> (Prionet SPAIN), que inclou a membres de <a href=\"https:\/\/asociacioncjd.org\/\" target=\"_blank\" rel=\"noopener noreferrer\">CJD Espa\u00f1a<\/a>, una associaci\u00f3 de pacients. &#8220;Amb ells, podem compartir experi\u00e8ncies i resultats cient\u00edfics que poden ajudar a altres pacients i les seves fam\u00edlies, aix\u00ed com entendre millor les malalties que pateixen&#8221;, diu Del R\u00edo.<\/p>\n<p>Aquesta recerca ha estat finan\u00e7ada per diverses fonts, incloent EiTB Maratoia (BioEF), FIS (ISCIII), MINECO, CIBERNED, La Marato de TV3, ICREA, i l&#8217;EU\u2019s Joint Programme &#8211; Neurodegenerative Disease Research (JPND). La tesi doctoral de l\u2019Andreu Matamoros ha estat finan\u00e7ada per la Fundaci\u00f3 Tatiana P\u00e9rez de Guzm\u00e1n el Bueno.<\/p>\n<p>&#8212;<\/p>\n<p><strong><em>Source article:<\/em> <\/strong>Matamoros-Angles, A., Gayosso, L.M., Richaud-Patin, Y. et al. Mol Neurobiol (2017). <a href=\"http:\/\/link.springer.com\/article\/10.1007\/s12035-017-0506-6\" target=\"_blank\" rel=\"noopener noreferrer\">iPS Cell Cultures from a Gerstmann-Str\u00e4ussler-Scheinker Patient with the Y218N PRNP Mutation Recapitulate tau Pathology<\/a>. <em>Molecular Neurobiology<\/em>, doi:10.1007\/s12035-017-0506-6<\/p>\n","protected":false},"excerpt":{"rendered":"<h3><img decoding=\"async\" data-src=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2017\/05\/Series008_ProjMax001.jpg\" alt=\"\" width=\"500\" height=\"500\" class=\"left size-full wp-image-29001 lazyload\" data-srcset=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2017\/05\/Series008_ProjMax001.jpg 500w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2017\/05\/Series008_ProjMax001-150x150.jpg 150w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2017\/05\/Series008_ProjMax001-300x300.jpg 300w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2017\/05\/Series008_ProjMax001-130x130.jpg 130w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2017\/05\/Series008_ProjMax001-45x45.jpg 45w\" data-sizes=\"(max-width: 500px) 100vw, 500px\" src=\"data:image\/svg+xml;base64,PHN2ZyB3aWR0aD0iMSIgaGVpZ2h0PSIxIiB4bWxucz0iaHR0cDovL3d3dy53My5vcmcvMjAwMC9zdmciPjwvc3ZnPg==\" style=\"--smush-placeholder-width: 500px; --smush-placeholder-aspect-ratio: 500\/500;\" \/>A partir de les c\u00e8l\u00b7lules d&#8217;un pacient amb una malaltia neurodegenerativa rara, la s\u00edndrome de Gerstmann-Str\u00e4ussler-Scheinker (GSS), investigadors de l&#8217;IBEC han aconseguit generar neurones que tamb\u00e9 presenten altres processos neurodegeneratius no relacionats amb la s\u00edndrome fins ara. <\/h3>\n<p>La impressionant imatge de l&#8217;esquerra mostra una massa de neurones derivades de c\u00e8l\u00b7lules mare pluripotents (iPS) afectades pel GSS, desenvolupades al laboratori de Jos\u00e9 Antonio Del R\u00edo.<\/p>\n","protected":false},"author":4,"featured_media":29001,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[96],"tags":[],"post_formats":[],"class_list":["post-29013","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-noticies-de-recerca"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.4 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Un model cel\u00b7lular per ajudar a estudiar la relaci\u00f3 entre malalties neurodegeneratives - Institute for Bioengineering of Catalonia<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/ibecbarcelona.eu\/ca\/un-model-cel\u00b7lular-per-ajudar-a-estudiar-la-relacio-entre-malalties-neurodegeneratives\/\" \/>\n<meta property=\"og:locale\" content=\"ca_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Un model cel\u00b7lular per ajudar a estudiar la relaci\u00f3 entre malalties neurodegeneratives - Institute for Bioengineering of Catalonia\" \/>\n<meta property=\"og:description\" content=\"A partir de les c\u00e8l\u00b7lules d&#039;un pacient amb una malaltia neurodegenerativa rara, la s\u00edndrome de Gerstmann-Str\u00e4ussler-Scheinker (GSS), investigadors de l&#039;IBEC han aconseguit generar neurones que tamb\u00e9 presenten altres processos neurodegeneratius no relacionats amb la s\u00edndrome fins ara.   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La impressionant imatge de l'esquerra mostra una massa de neurones derivades de c\u00e8l\u00b7lules mare pluripotents (iPS) afectades pel GSS, desenvolupades al laboratori de Jos\u00e9 Antonio Del R\u00edo.","og_url":"https:\/\/ibecbarcelona.eu\/ca\/un-model-cel\u00b7lular-per-ajudar-a-estudiar-la-relacio-entre-malalties-neurodegeneratives\/","og_site_name":"Institute for Bioengineering of Catalonia","article_published_time":"2017-05-05T08:14:45+00:00","article_modified_time":"2017-05-15T09:51:02+00:00","og_image":[{"width":500,"height":500,"url":"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2017\/05\/Series008_ProjMax001.jpg","type":"image\/jpeg"}],"author":"\u00c0ngels L\u00f3pez","twitter_card":"summary_large_image","twitter_misc":{"Escrit per":"\u00c0ngels L\u00f3pez","Temps estimat de lectura":"4 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