{"id":39248,"date":"2015-10-26T00:00:00","date_gmt":"2015-10-25T23:00:00","guid":{"rendered":"http:\/\/www.pcb.ub.edu\/\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\/"},"modified":"2020-11-16T16:23:13","modified_gmt":"2020-11-16T15:23:13","slug":"una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo","status":"publish","type":"post","link":"https:\/\/www.pcb.ub.edu\/es\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\/","title":{"rendered":"Una investigaci\u00f3n aplica la tecnolog\u00eda de c\u00e9lulas iPS al estudio del s\u00edndrome de Sanfilippo"},"content":{"rendered":"<p>A partir de les c\u00e8l\u00b7lules iPS, els autors de la recerca \u2013publicada a la revista\u00a0Stem Cell Reports\u00a0(<a href=\"http:\/\/www.ncbi.nlm.nih.gov\/pubmed\/26411903\" target=\"_blank\" rel=\"noopener noreferrer\">doi: 10.1016\/j.stemcr.2015.08.016<\/a>)\u2013 han generat neurones madures que reprodueixen les caracter\u00edstiques principals de la malaltia, les quals no s&#8217;observen, en canvi, en les neurones espec\u00edfiques de pacients a les quals s&#8217;ha introdu\u00eft l&#8217;ADNc salvatge del gen\u00a0HGSNAT.<\/p>\n<p>A m\u00e9s, les xarxes neuronals organitzades in vitro a partir de neurones madures derivades de pacients mostren defectes primerencs en l&#8217;activitat neuronal, degradaci\u00f3 de la xarxa neuronal i alteracions de connectivitat. Aquestes troballes constaten la import\u00e0ncia de la tecnologia basada en c\u00e8l\u00b7lules iPS per identificar fenotips funcionals primerencs; la qual cosa, al seu torn, pot aclarir els mecanismes patol\u00f2gics que causen la s\u00edndrome de Sanfilippo.<\/p>\n<p>Aix\u00ed mateix, aquesta tecnologia pot ser \u00fatil per analitzar compostos i opcions terap\u00e8utiques que permetin evitar el comen\u00e7ament de la neurodegeneraci\u00f3. En el treball hi han participat els investigadors de la Universitat de Barcelona Daniel Grinberg, Isaac Canals i Llu\u00efsa Vilageliu, del Departament de Gen\u00e8tica i membres de l&#8217;IBUB\u00a0i del Centre d&#8217;Investigaci\u00f3 Biom\u00e8dica en Xarxa de Malalties Rares (CIBERER). Tamb\u00e9 han col\u00b7laborat en la recerca Jordi Soriano i Javier G. Orlandi, de la Facultat de F\u00edsica, i el grup que lidera \u00c1ngel Raya, director del Centre de Medicina Regenerativa de Barcelona (CMBR) i investigador de l&#8217;IBEC, centre adscrit a la Universitat de Barcelona.<br \/>\n\u00a0<\/p>\n<p><strong>Referencia del art\u00edculo:<\/strong><\/p>\n<p>Canals, I.; Soriano, J.; Orlandi, J. G.; Torrent, R.; Richaud-Patin, Y.; Jim\u00e9nez-Delgado, S.; Merlin, S.; Follenzi, A.; Consiglio, A.; Vilageliu, Ll.; Grinberg, D.; Raya, A.\u00a0<a href=\"http:\/\/www.cell.com\/stem-cell-reports\/abstract\/S2213-6711%2815%2900250-7\" target=\"_blank\" rel=\"noopener noreferrer\">\u00abActivity and high-order effective connectivity alterations in Sanfilippo C patient-specific neuronal networks\u00bb<\/a>.\u00a0Stem Cell Reports, octubre de 2015. Doi: 10.1016\/j.stemcr.2015.08.016<\/p>\n","protected":false},"excerpt":{"rendered":"<p>El s\u00edndrome de Sanfilippo tipo C es una enfermedad de ac\u00famulo lisosomal debida a mutaciones en el gen\u00a0HGSNAT\u00a0que conllevan una neurodegeneraci\u00f3n progresiva. Ahora, en un estudio en el que han participado investigadores del\u00a0<a href=\"http:\/\/www.pcb.ub.edu\/portal\/es\/cerca?p_p_id=cercador_WAR_empleatsempresesportlet&amp;p_p_lifecycle=0&amp;p_p_state=maximized&amp;p_p_mode=view&amp;p_p_col_id=column-1&amp;p_p_col_count=1&amp;_cercador_WAR_empleatsempresesportlet_jspPage=%2Fhtml%2Fcommon%2Fdetall-empresa.jsp&amp;_cercador_WAR_empleatsempresesportlet_empresaId=ENT_002730&amp;_cercador_WAR_empleatsempresesportlet_redirect=%2Fportal%2Fes%2Fcerca%3Fp_p_id%3Dcercador_WAR_empleatsempresesportlet%26p_p_lifecycle%3D0%26p_p_mode%3Dview%26_cercador_WAR_empleatsempresesportlet_keywords%3DIBUB%26_cercador_WAR_empleatsempresesportlet_tab%3D1\" target=\"_blank\" rel=\"noopener noreferrer\">Instituto de Biomedicina de la Universitat de Barcelona (IBUB)<\/a>\u00a0i del\u00a0<a href=\"http:\/\/www.pcb.ub.edu\/portal\/es\/cerca?p_p_id=cercador_WAR_empleatsempresesportlet&amp;p_p_lifecycle=0&amp;p_p_state=maximized&amp;p_p_mode=view&amp;p_p_col_id=column-1&amp;p_p_col_count=1&amp;_cercador_WAR_empleatsempresesportlet_jspPage=%2Fhtml%2Fcommon%2Fdetall-empresa.jsp&amp;_cercador_WAR_empleatsempresesportlet_empresaId=ENT_000134&amp;_cercador_WAR_empleatsempresesportlet_redirect=%2Fportal%2Fca%2Fcerca%3Fp_p_id%3Dcercador_WAR_empleatsempresesportlet%26p_p_lifecycle%3D0%26p_p_mode%3Dview%26_cercador_WAR_empleatsempresesportlet_keywords%3Dibec%26_cercador_WAR_empleatsempresesportlet_tab%3D1\" target=\"_blank\" rel=\"noopener noreferrer\">Instituto de Bioingenier\u00eda de Catalu\u00f1a (IBEC)<\/a>\u00a0en el Parc Cient\u00edfic de Barcelona,\u00a0se han generado c\u00e9lulas madre pluripotentes inducidas (iPS) derivadas de dos pacientes con esta patologia que pueden ayudar a buscar nuevas opciones terap\u00e9uticas \u00a0y a aclarar los mecanismos patol\u00f3gicos que la causan.<\/p>\n","protected":false},"author":1,"featured_media":27448,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"inline_featured_image":false,"footnotes":""},"categories":[9],"tags":[],"class_list":["post-39248","post","type-post","status-publish","format-standard","has-post-thumbnail","category-sin-categorizar"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.0 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Una investigaci\u00f3n aplica la tecnolog\u00eda de c\u00e9lulas iPS al estudio del s\u00edndrome de Sanfilippo - Parc Cient\u00edfic de Barcelona<\/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:\/\/www.pcb.ub.edu\/es\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\/\" \/>\n<meta property=\"og:locale\" content=\"es_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Una investigaci\u00f3n aplica la tecnolog\u00eda de c\u00e9lulas iPS al estudio del s\u00edndrome de Sanfilippo - Parc Cient\u00edfic de Barcelona\" \/>\n<meta property=\"og:description\" content=\"El s\u00edndrome de Sanfilippo tipo C es una enfermedad de ac\u00famulo lisosomal debida a mutaciones en el gen\u00a0HGSNAT\u00a0que conllevan una neurodegeneraci\u00f3n progresiva. Ahora, en un estudio en el que han participado investigadores del\u00a0Instituto de Biomedicina de la Universitat de Barcelona (IBUB)\u00a0i del\u00a0Instituto de Bioingenier\u00eda de Catalu\u00f1a (IBEC)\u00a0en el Parc Cient\u00edfic de Barcelona,\u00a0se han generado c\u00e9lulas madre pluripotentes inducidas (iPS) derivadas de dos pacientes con esta patologia que pueden ayudar a buscar nuevas opciones terap\u00e9uticas \u00a0y a aclarar los mecanismos patol\u00f3gicos que la causan.\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.pcb.ub.edu\/es\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\/\" \/>\n<meta property=\"og:site_name\" content=\"Parc Cient\u00edfic de Barcelona\" \/>\n<meta property=\"article:published_time\" content=\"2015-10-25T23:00:00+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2020-11-16T15:23:13+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.pcb.ub.edu\/wp-content\/uploads\/2015\/10\/16049227565fa92d84ce931.jpeg\" \/>\n\t<meta property=\"og:image:width\" content=\"500\" \/>\n\t<meta property=\"og:image:height\" content=\"375\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"adminpcb\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Escrito por\" \/>\n\t<meta name=\"twitter:data1\" content=\"adminpcb\" \/>\n\t<meta name=\"twitter:label2\" content=\"Tiempo de lectura\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minuto\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/www.pcb.ub.edu\\\/es\\\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/www.pcb.ub.edu\\\/es\\\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\\\/\"},\"author\":{\"name\":\"adminpcb\",\"@id\":\"https:\\\/\\\/www.pcb.ub.edu\\\/es\\\/#\\\/schema\\\/person\\\/d13cab05cd0a75b17fddca8496481021\"},\"headline\":\"Una investigaci\u00f3n aplica la tecnolog\u00eda de c\u00e9lulas iPS al estudio del s\u00edndrome de Sanfilippo\",\"datePublished\":\"2015-10-25T23:00:00+00:00\",\"dateModified\":\"2020-11-16T15:23:13+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/www.pcb.ub.edu\\\/es\\\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\\\/\"},\"wordCount\":310,\"image\":{\"@id\":\"https:\\\/\\\/www.pcb.ub.edu\\\/es\\\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\\\/#primaryimage\"},\"thumbnailUrl\":\"https:\\\/\\\/www.pcb.ub.edu\\\/wp-content\\\/uploads\\\/2015\\\/10\\\/16049227565fa92d84ce931.jpeg\",\"articleSection\":[\"Sin categorizar\"],\"inLanguage\":\"es\"},{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/www.pcb.ub.edu\\\/es\\\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\\\/\",\"url\":\"https:\\\/\\\/www.pcb.ub.edu\\\/es\\\/una-investigacion-aplica-la-tecnologia-de-celulas-ips-al-estudio-del-sindrome-de-sanfilippo\\\/\",\"name\":\"Una investigaci\u00f3n aplica la tecnolog\u00eda de c\u00e9lulas iPS al estudio del s\u00edndrome de Sanfilippo - 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