{"id":93285,"date":"2022-02-24T09:41:00","date_gmt":"2022-02-24T08:41:00","guid":{"rendered":"https:\/\/testweb.ibecbarcelona.eu\/?p=93285"},"modified":"2022-04-04T09:52:17","modified_gmt":"2022-04-04T07:52:17","slug":"la-falta-de-proteina-prionica-cellular-pot-provocar-simptomes-depilepsia-i-deficits-daprenentatge","status":"publish","type":"post","link":"https:\/\/ibecbarcelona.eu\/ca\/la-falta-de-proteina-prionica-cellular-pot-provocar-simptomes-depilepsia-i-deficits-daprenentatge\/","title":{"rendered":"La falta de prote\u00efna pri\u00f2nica cel\u00b7lular pot provocar s\u00edmptomes d’epil\u00e8psia i d\u00e8ficits d’aprenentatge"},"content":{"rendered":"\n

Investigadors de l’IBEC, en col\u00b7laboraci\u00f3 amb hospitals i socis internacionals, han observat que la defici\u00e8ncia de la prote\u00efna pri\u00f2nica cel\u00b7lular provoca d\u00e8ficits de comportament, defici\u00e8ncies en l’aprenentatge i un augment de l’excitabilitat del cervell semblant a l’epil\u00e8psia, en ratolins.<\/h4>\n\n\n\n
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La prote\u00efna pri\u00f2nica cel\u00b7lular (PrPC), una mol\u00e8cula situada a la superf\u00edcie de les c\u00e8l\u00b7lules neuronals, es coneix per trobar-se desequilibrada en pacients humans que pateixen malalties neurodegeneratives com l’Alzheimer o la malaltia de Parkinson. Tanmateix, un millor coneixement sobre la participaci\u00f3 de PrPC en aquestes malalties, contrasta amb les dades sobre el seu rol natural i promotor de la salut. Aix\u00f2 vol dir que, mentre que en alguns estudis aquesta prote\u00efna s’ha descrit com a protectora per al cervell, en altres estudis l’expressi\u00f3 de PrPC s’ha associat a una major susceptibilitat a la neurotoxicitat.<\/p>\n<\/div><\/div>\n\n\n\n

Aquestes inconsist\u00e8ncies es deuen principalment a l’abs\u00e8ncia de models de ratolins adequats i ben caracteritzats. Per aportar una mica m\u00e9s de llum a la funci\u00f3 real de PrPC en fisiologia neuronal, investigadors de l’Institut de Bioenginyeria de Catalunya (IBEC), dirigits per Jos\u00e9 Antonio del R\u00edo, catedr\u00e0tic de la Universitat de Barcelona i l\u00edder del grup de l’IBEC han treballat intensament en els \u00faltims cinc anys en un nou ratol\u00ed transg\u00e8nic sense PrPC generat amb la tecnologia TALEN, i n’informen ara a la revista BCM Biology, amb resultats sorprenents.<\/p>\n\n\n\n

Cient\u00edfics del grup de Neurobiotecnologia Molecular i Cel\u00b7lular<\/a> de l’IBEC, en col\u00b7laboraci\u00f3 amb l’Hospital Universitari de Zuric, la Universitat de Barcelona i la Universitat Pablo de Olavide de Sevilla (Espanya), han descobert que l’abs\u00e8ncia de PrPC perjudica les funcions cognitives com ara l’aprenentatge associatiu i la mem\u00f2ria, i produeix un comportament semblant a l’ansietat. Els experts tamb\u00e9 han trobat que la defici\u00e8ncia de PrPC comporta una major susceptibilitat a les convulsions d’epil\u00e8psia en aquests ratolins.<\/p>\n\n\n\n

Els investigadors han treballat durant d\u00e8cades amb ratolins modificats amb PrPC, sense con\u00e8ixer l’impacte en la seva salut, degut a alguns inconvenients t\u00e8cnics que presentaven els models desenvolupats anteriorment. Utilitzant uns ratolins eliminadors de PrPC generats per TALEN, ara hem descobert que els animals que no expressen la prote\u00efna presenten s\u00edmptomes semblants a l’epil\u00e8psia i una defici\u00e8ncia en l’aprenentatge i de la mem\u00f2ria que reforcen el paper de la prote\u00efna en aquests processos, i que poden ajudar a comprendre els efectes de la seva disminuci\u00f3 observada en algunes malalties neurodegeneratives en les persones.\u00a0<\/p>

<\/p>Jos\u00e9 Antonio del R\u00edo, l\u00edder de grup a l’IBEC\u00a0<\/cite><\/blockquote>\n\n\n\n

En el seu estudi, els investigadors van realitzar un conjunt complet de proves de condicionament conductual i operant per avaluar la mem\u00f2ria i les capacitats d’aprenentatge dels ratolins, en paral\u00b7lel amb l’RNA-seq, l’an\u00e0lisi de la xarxa de neurones in vitro<\/em> i dades electrofisiol\u00f2giques in vivo<\/em>. Els resultats van mostrar una clara disminuci\u00f3 de la motilitat, defici\u00e8ncies en l’aprenentatge del condicionament operant i un comportament relacionat amb l’ansietat en els animals sense PrPC. <\/p>\n\n\n\n

Els resultats demostren que la prote\u00efna PrPC afavoreix la formaci\u00f3 de xarxes neuronals i la connectivitat, interv\u00e9 en la funci\u00f3 sin\u00e0ptica, i protegeix tamb\u00e9 de l’excitotoxicitat. La supressi\u00f3 de PrPC pot ser la base d’un cervell susceptible a l’epil\u00e8psia, que no pot realitzar tasques cognitives gaire exigents, com ara l’aprenentatge associatiu i els comportaments semblants a l’ansietat. <\/p>\n\n\n\n

Aquest estudi realitzat els darrers anys ha comptat amb el suport de La Caixa Research, del Projecte PRPSEM del Ministeri de Ci\u00e8ncia i Innovaci\u00f3, del CIBERNED, del Programa CERCA i de la Comissi\u00f3 d’Universitats i Recerca del Departament d’Innovaci\u00f3, Universitats i Empresa de la Generalitat de Catalunya.  <\/p>\n\n\n\n

<\/div>\n\n\n\n

Article de refer\u00e8ncia: <\/strong>Matamoros-Angles et al. Analysis of co-isogenic prion protein deficient mice reveals behavioral deficits, learning impairment, and enhanced hippocampal excitability<\/a>. BMC Biology 2022.\u00a0<\/p>\n","protected":false},"excerpt":{"rendered":"

Investigadors de l’IBEC, en col\u00b7laboraci\u00f3 amb hospitals i socis internacionals, han observat que la defici\u00e8ncia de la prote\u00efna pri\u00f2nica cel\u00b7lular provoca d\u00e8ficits de comportament, defici\u00e8ncies en l’aprenentatge i un augment de l’excitabilitat del cervell semblant a l’epil\u00e8psia, en ratolins. <\/h3>\n","protected":false},"author":4,"featured_media":93283,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[96],"tags":[1132,1581],"post_formats":[],"class_list":["post-93285","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-noticies-de-recerca","tag-jose-antonio-del-rio-ca","tag-molecular-and-cellular-neurobiotechnology-group-ca"],"yoast_head":"\nLa falta de prote\u00efna pri\u00f2nica cel\u00b7lular pot provocar s\u00edmptomes d'epil\u00e8psia i d\u00e8ficits d'aprenentatge - 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\/la-falta-de-proteina-prionica-cellular-pot-provocar-simptomes-depilepsia-i-deficits-daprenentatge\/\" \/>\n<meta property=\"og:locale\" content=\"ca_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"La falta de prote\u00efna pri\u00f2nica cel\u00b7lular pot provocar s\u00edmptomes d'epil\u00e8psia i d\u00e8ficits d'aprenentatge - 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