{"id":18919,"date":"2021-09-07T10:00:40","date_gmt":"2021-09-07T08:00:40","guid":{"rendered":"https:\/\/idibell.cat\/?p=18919"},"modified":"2021-09-07T10:02:32","modified_gmt":"2021-09-07T08:02:32","slug":"un-compost-natural-designat-medicament-orfe-per-a-la-cistinuria","status":"publish","type":"post","link":"https:\/\/idibell.cat\/2021\/09\/un-compost-natural-designat-medicament-orfe-per-a-la-cistinuria\/","title":{"rendered":"Un compost natural, designat medicament orfe per a la cistin\u00faria"},"content":{"rendered":"<p>Un compost natural amb propietats antioxidants, la <strong>L-ergotioneina<\/strong>, ha estat designat com a medicament orfe per al tractament de la cistin\u00faria per l&#8217;Ag\u00e8ncia Europea de Medicaments (EMA). Els estudis realitzats han demostrat en un model mur\u00ed d&#8217;aquesta malaltia rara que la L-ergotioneina podria prevenir la formaci\u00f3 de les pedres de cistina en el rony\u00f3 i les vies urin\u00e0ries caracter\u00edstiques d&#8217;aquesta patologia o retardar la seva aparici\u00f3.<\/p>\n<p>La investigaci\u00f3 que ha possibilitat arribar a la designaci\u00f3 de medicament orfe per a aquesta mol\u00e8cula, ha estat desenvolupada pel <a href=\"https:\/\/idibell.cat\/recerca\/area-de-medicina-translacional\/programa-de-gens-malaltia-i-terapia\/genetica-molecular-humana\/\">grup de recerca en Gen\u00e8tica Molecular Humana<\/a> liderat per la Dra. Virginia Nunes, investigadora de l&#8217;IDIBELL i de la <a href=\"https:\/\/www.ub.edu\/web\/portal\/ca\/\">Universitat de Barcelona<\/a>, juntament amb el Dr. Miguel L\u00f3pez de Heredia de el Centre d&#8217;Investigaci\u00f3 Biom\u00e8dica en Xarxa de Malalties Rares (<a href=\"https:\/\/www.ciberer.es\/\">CIBERER<\/a>) des d&#8217;on s&#8217;ha promogut la petici\u00f3.<\/p>\n<p>La <strong>cistin\u00faria<\/strong> \u00e9s una malaltia gen\u00e8tica heredit\u00e0ria que afecta 1 de cada 7.000 persones. Es caracteritza per l&#8217;aparici\u00f3 recurrent de pedres de cistina en les vies urin\u00e0ries i rony\u00f3 durant tota la vida del pacient, el que provoca un deteriorament de la seva qualitat de vida.<\/p>\n<p>Els tractaments preventius actuals s\u00f3n en molts casos complexos, poc efectius i tenen efectes adversos, el que provoca que molts afectats els abandonin. Per tot aix\u00f2, els pacients passen per freq\u00fcents processos quir\u00fargics per extreure les pedres que es formen.<\/p>\n<p>La <strong>L-ergotioneina \u00e9s un compost natural amb propietats antioxidants<\/strong> identificat fa 100 anys que, a difer\u00e8ncia dels tractaments actuals contra la malaltia, mostra una baixa toxicitat, el que podria permetre el tractament cr\u00f2nic dels pacients amb cistin\u00faria. En els models murins utilitzats pels investigadors, l&#8217;administraci\u00f3 d&#8217;aquest compost ha redu\u00eft la formaci\u00f3 de pedres de manera segura i efica\u00e7.<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/p>\n<p><em>L\u2019Institut d&#8217;Investigaci\u00f3 Biom\u00e8dica de Bellvitge (IDIBELL) \u00e9s un centre de recerca en biomedicina creat l\u2019any 2004. Est\u00e0 participat per l&#8217;Hospital Universitari de Bellvitge i l\u2019Hospital de Viladecans de l&#8217;Institut Catal\u00e0 de la Salut, l&#8217;Institut Catal\u00e0 d&#8217;Oncologia, la Universitat de Barcelona i l\u2019Ajuntament de l\u2019Hospitalet de Llobregat.<\/em><\/p>\n<p><em>L\u2019IDIBELL \u00e9s membre del Campus d&#8217;Excel\u00b7l\u00e8ncia Internacional de la Universitat de Barcelona HUBc i forma part de la instituci\u00f3 CERCA de la Generalitat de Catalunya. L&#8217;any 2009 es va convertir en un dels cinc primers centres d\u2019investigaci\u00f3 espanyols acreditats com a institut d\u2019investigaci\u00f3 sanit\u00e0ria per l\u2019Instituto de Salud Carlos III. A m\u00e9s, forma part del programa \u201cHR Excellence in Research\u201d de la Uni\u00f3 Europea i \u00e9s membre de EATRIS i REGIC. Des de l\u2019any 2018, l\u2019IDIBELL \u00e9s un Centro Acreditado de la Fundaci\u00f3n Cient\u00edfica AECC (FCAECC).<\/em><\/p>\n","protected":false},"excerpt":{"rendered":"<p>La cistin\u00faria \u00e9s una malaltia rara caracteritzada per la formaci\u00f3 de pedres de cistina en el rony\u00f3 i les vies urin\u00e0ries. Aquest compost podria prevenir o retardar l&#8217;aparici\u00f3 de les pedres de cistina.<\/p>\n","protected":false},"author":8,"featured_media":18920,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"site-sidebar-layout":"default","site-content-layout":"default","ast-site-content-layout":"","site-content-style":"default","site-sidebar-style":"default","ast-global-header-display":"","ast-banner-title-visibility":"","ast-main-header-display":"","ast-hfb-above-header-display":"","ast-hfb-below-header-display":"","ast-hfb-mobile-header-display":"","site-post-title":"","ast-breadcrumbs-content":"","ast-featured-img":"","footer-sml-layout":"","theme-transparent-header-meta":"default","adv-header-id-meta":"","stick-header-meta":"default","header-above-stick-meta":"","header-main-stick-meta":"","header-below-stick-meta":"","astra-migrate-meta-layouts":"default","ast-page-background-enabled":"default","ast-page-background-meta":{"desktop":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}},"ast-content-background-meta":{"desktop":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"tablet":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""},"mobile":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-opacity":"","overlay-gradient":""}},"footnotes":""},"categories":[396,334,464],"tags":[],"class_list":["post-18919","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-genetica-molecular-humana","category-medicina-translacional","category-p-de-gens-malaltia-i-terapia"],"publishpress_future_action":{"enabled":false,"date":"2024-12-29 08:51:30","action":"change-status","newStatus":"draft","terms":[],"taxonomy":"category"},"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"_links":{"self":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/18919","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/comments?post=18919"}],"version-history":[{"count":1,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/18919\/revisions"}],"predecessor-version":[{"id":18921,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/18919\/revisions\/18921"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/media\/18920"}],"wp:attachment":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/media?parent=18919"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/categories?post=18919"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/tags?post=18919"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}