{"id":17999,"date":"2015-07-09T12:55:56","date_gmt":"2015-07-09T12:55:56","guid":{"rendered":"http:\/\/ibecbarcelona.eu\/?p=17999"},"modified":"2016-06-11T18:24:07","modified_gmt":"2016-06-11T18:24:07","slug":"cientifics-de-libec-associen-els-atacs-epileptics-durant-les-malalties-neurodegeneratives-a-la-perdua-de-funcio-de-la-proteina-prionica-cel%c2%b7lular","status":"publish","type":"post","link":"https:\/\/ibecbarcelona.eu\/ca\/cientifics-de-libec-associen-els-atacs-epileptics-durant-les-malalties-neurodegeneratives-a-la-perdua-de-funcio-de-la-proteina-prionica-cel%c2%b7lular\/","title":{"rendered":"Cient\u00edfics de l&#8217;IBEC associen els atacs epil\u00e8ptics durant les malalties neurodegeneratives a la p\u00e8rdua de funci\u00f3 de la prote\u00efna pri\u00f2nica cel\u00b7lular"},"content":{"rendered":"<p>i els seus col\u00b7laboradors han publicat les troballes que podrien conduir a una millor comprensi\u00f3 de les malalties neurodegeneratives r\u00e0pides que impliquen atacs epil\u00e8ptics, com la malaltia de Creutzfeldt-Jacob (ECJ)<\/p>\n<h6 class=\"caption\" style=\"float: left; width: 310px; padding: 0px 20px 10px 0px;\">Imatge: Secci\u00f3 de l&#8217;hipocamp de ratol\u00ed knock-out per a dues prote\u00efnes pri\u00f2niques que sobre expressen una forma truncada de prote\u00efna pri\u00f2nica DF35. A l&#8217;esquerra, una tinci\u00f3 de Nissl d&#8217;un ratol\u00ed no-tractat mostrant l&#8217;estructura t\u00edpica de les capes piramidals de CA1-CA3. A la dreta, una tinci\u00f3 de FluoroJade B 24h despr\u00e9s d&#8217;un tractament amb KA. La tinci\u00f3 marca espec\u00edficament la degeneraci\u00f3 neuronal, de manera que el patr\u00f3 neurodegeneratiu que es veu est\u00e0 relacionat \u00fanicament amb el tractament KA, demostrant aix\u00ed el paper neuroprotector essencial de la prote\u00efna pri\u00f2nica<\/h6>\n<p>&#8220;Vam desenvolupar una an\u00e0lisi comparativa de la susceptibilitat de convulsi\u00f3 utilitzant ratolins amb sis her\u00e8ncies gen\u00e8tiques diferents i diversos experiments in vitro&#8221;, diu Jos\u00e9 Antonio del R\u00edo, responsable del grup de Neurobiotecnologia Molecular i Cel\u00b7lular de l&#8217;IBEC. &#8220;A m\u00e9s, vam demostrar que els ratolins als quals els faltava PrP<sup>C<\/sup> mostraven un increment de susceptibilitat als episodis epil\u00e8ptics que porten a la mort cel\u00b7lular en la regi\u00f3 de l&#8217;hipocamp, recolzant la idea que la prote\u00efna est\u00e0 implicada en la neuroprotecci\u00f3 contra convulsions, i que participa activament en l&#8217;increment de la resposta epil\u00e8ptica observada en ratolins, independentment de la seva c\u00e0rrega gen\u00e8tica, als que els faltava&#8221;.<\/p>\n<p>La prote\u00efna PrP<sup>C<\/sup> es troba de forma natural en el cervell sa i, en menor quantitat, quan el cervell est\u00e0 afectat per malalties com l&#8217;encefalopatia espongiforme bovina o la dem\u00e8ncia humana r\u00e0pidament progressiva. Des de fa temps se li reconeix un efecte neuroprotector, i els investigadors de l&#8217;IBEC han arribat a la conclusi\u00f3 que els s\u00edmptomes de les convulsions en les malalties neurodegeneratives estan associats a la p\u00e8rdua de la seva funci\u00f3.<\/p>\n<p>L&#8217;estudi, que tamb\u00e9 ha involucrat a investigadors de la UB, l&#8217;IDIBELL, CIBERNED, CISA-INIA, DZNE (Alemanya) i SISSA (It\u00e0lia), tamb\u00e9 han desvetllat que hi ha altres factors paral\u00b7lels a la supressi\u00f3 de PrP<sup>C<\/sup>, com per exemple, la c\u00e0rrega gen\u00e8tica d&#8217;alguns dels ratolins utilitzats per a l&#8217;an\u00e0lisi de PrP<sup>C<\/sup>, o la pres\u00e8ncia de l&#8217;anomenat &#8216;PrNP-flanking gens&#8217; en els ratolins knockout per PrP<sup>C<\/sup>, que poden contribuir a la susceptibilitat d&#8217;atacs epil\u00e8ptics en abs\u00e8ncia de PrP<sup>C<\/sup>.<\/p>\n<p>&#8220;A la neurodegeneraci\u00f3 trobada en casos de dem\u00e8ncia r\u00e0pidament progressiva, CJD, Alzheimer o dem\u00e8ncia amb cossos de Lewy, el temps de superviv\u00e8ncia des del diagn\u00f2stic cl\u00ednic fins a la mort \u00e9s molt curt, sent la tremolor i les convulsions epil\u00e8ptiques caracter\u00edstiques comunes d&#8217;algunes d&#8217;aquestes dem\u00e8ncies, &#8220;diu del R\u00edo. &#8220;Amb aquestes noves descobertes, estem una mica m\u00e9s a prop de comprendre m\u00e9s sobre les malalties neurodegeneratives que involucren convulsions epil\u00e8ptiques, el que significa estar un pas m\u00e9s a prop de desenvolupar noves ter\u00e0pies o eines de diagn\u00f2stic.&#8221;<\/p>\n<p>&#8212; <strong>Refer\u00e8ncia:<\/strong> Patricia Carulla, Franc Llorens, Andreu Matamoros-Angles, Patricia Aguilar-Calvo, Juan Carlos Espinosa, Rosalina Gav\u00edn, Isidre Ferrer, Giuseppe Legname, Juan Maria Torres &amp; Jos\u00e9 A. del R\u00edo (2015). <a href=\"http:\/\/www.nature.com\/srep\/2015\/150709\/srep11971\/full\/srep11971.html\" target=\"_blank\">Involvement of PrP<sup>C<\/sup> in kainate-induced excitotoxicity in several mouse strains<\/a>.\u00a0<em>Scientific Reports<\/em>, 5, 11971<\/p>\n","protected":false},"excerpt":{"rendered":"<h3><img decoding=\"async\" data-src=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2015\/07\/FINAL-IMAGE_del-rio-scirep1.jpg\" alt=\"\" width=\"400\" height=\"429\" class=\"left size-full wp-image-16965 lazyload\" src=\"data:image\/svg+xml;base64,PHN2ZyB3aWR0aD0iMSIgaGVpZ2h0PSIxIiB4bWxucz0iaHR0cDovL3d3dy53My5vcmcvMjAwMC9zdmciPjwvc3ZnPg==\" style=\"--smush-placeholder-width: 400px; --smush-placeholder-aspect-ratio: 400\/429;\" \/>Investigadors de l&#8217;Institut de Bioenginyeria de Catalunya (IBEC) han revelat que la prote\u00efna pri\u00f2nica cel\u00b7lular (PrPC) -associada a una gran quantitat de funcions biol\u00f2giques, com la proliferaci\u00f3 cel\u00b7lular, la diferenciaci\u00f3 i la senyalitzaci\u00f3- juga un paper determinant en l&#8217;epil\u00e8psia.<br \/>\n<\/h3>\n<p>A l&#8217;\u00faltima edici\u00f3 de Scientific Reports, revista del grup Nature, el grup de Neurobiotecnologia Molecular i Cel\u00b7lular de l&#8217;IBEC<\/p>\n","protected":false},"author":4,"featured_media":17959,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[96],"tags":[],"post_formats":[],"class_list":["post-17999","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 v27.5 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Cient\u00edfics de l&#039;IBEC associen els atacs epil\u00e8ptics durant les malalties neurodegeneratives a la p\u00e8rdua de funci\u00f3 de la prote\u00efna pri\u00f2nica cel\u00b7lular - 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\/cientifics-de-libec-associen-els-atacs-epileptics-durant-les-malalties-neurodegeneratives-a-la-perdua-de-funcio-de-la-proteina-prionica-cel\u00b7lular\/\" \/>\n<meta property=\"og:locale\" content=\"ca_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Cient\u00edfics de l&#039;IBEC associen els atacs epil\u00e8ptics durant les malalties neurodegeneratives a la p\u00e8rdua de funci\u00f3 de la prote\u00efna pri\u00f2nica cel\u00b7lular - Institute for Bioengineering of Catalonia\" \/>\n<meta property=\"og:description\" content=\"Investigadors de l&#039;Institut de Bioenginyeria de Catalunya (IBEC) han revelat que la prote\u00efna pri\u00f2nica cel\u00b7lular (PrPC) -associada a una gran quantitat de funcions biol\u00f2giques, com la proliferaci\u00f3 cel\u00b7lular, la diferenciaci\u00f3 i la senyalitzaci\u00f3- juga un paper determinant en l&#039;epil\u00e8psia.  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