{"id":8267,"date":"2024-04-24T13:05:28","date_gmt":"2024-04-24T11:05:28","guid":{"rendered":"https:\/\/www.iqac.csic.es\/un-complement-alimentari-te-efectes-beneficiosos-en-la-malaltia-de-niemann-pick-tipus-c\/"},"modified":"2026-06-16T10:47:24","modified_gmt":"2026-06-16T08:47:24","slug":"un-complement-alimentari-te-efectes-beneficiosos-en-la-malaltia-de-niemann-pick-tipus-c","status":"publish","type":"post","link":"https:\/\/www.iqac.csic.es\/ca\/un-complement-alimentari-te-efectes-beneficiosos-en-la-malaltia-de-niemann-pick-tipus-c\/","title":{"rendered":"Un complement alimentari t\u00e9 efectes beneficiosos en la malaltia de Niemann-Pick tipus C"},"content":{"rendered":"<p><strong>La malaltia de Niemann-Pick tipus C<\/strong> \u00e9s un trastorn hereditari per al qual no hi ha tractament, i que est\u00e0 causat per nivells baixos de les prote\u00efnes NPC1 i NPC2, que regulen el colesterol als lisosomes (org\u00e0nuls de les c\u00e8l\u00b7lules). En conseq\u00fc\u00e8ncia, el cos no pot descompondre apropiadament el colesterol, que s&#8217;acumula a les c\u00e8l\u00b7lules i causa, alhora, efectes neurodegeneratius que afecten principalment el cerebel amb un impacte est\u00e8s a \u00f2rgans viscerals com el fetge.<\/p>\n<p>Aquest treball, dut a terme pel grup \u201cRegulaci\u00f3 mitocondrial de la mort cel\u00b7lular\u201d de <a href=\"https:\/\/www.iibb.csic.es\/es\" target=\"_blank\" rel=\"noopener\">l&#8217;IIBB-CSIC<\/a>, de <a href=\"https:\/\/www.clinicbarcelona.org\/idibaps\" target=\"_blank\" rel=\"noopener\">l&#8217;IDIBAPS<\/a> i del <a href=\"https:\/\/www.ciberehd.org\/\" target=\"_blank\" rel=\"noopener\">CIBEREHD<\/a>, amb <strong>Leire Goicoechea<\/strong> com a primera autora i dirigit per <strong>Carmen Garc\u00eda-Ruiz<\/strong> i<strong> Jos\u00e9 C Fern\u00e1ndez-Checa, <\/strong>revela que la <strong>S-adenosil-L-metionina (SAM)<\/strong> podria ser una nova estrat\u00e8gia per al tractament d\u2019aquesta malaltia. La SAM \u00e9s una subst\u00e0ncia que produeix l&#8217;organisme de manera natural i que participa en funcions essencials. Per\u00f2 tamb\u00e9 se sintetitza i comercialitza com a complement, i s&#8217;utilitza actualment per al tractament de malalties psiqui\u00e0triques, neurol\u00f2giques i hep\u00e0tiques.<\/p>\n<p>En aquest treball ha investigat el paper i els nivells de la mol\u00e8cula SAM en ratolins <em>NPC1-\/-,<\/em> un model mur\u00ed que reprodueix les caracter\u00edstiques de la malaltia NPC en humans.<\/p>\n<p>L\u00b4estudi ha comptat tamb\u00e9 amb la col\u00b7laboraci\u00f3 dels equips de <strong>Josefina Casas<\/strong> i <strong>Gemma Fabri\u00e0s<\/strong>, de l&#8217;IQAC-CSIC, i s\u00b4ha realitzat gr\u00e0cies, en part, al finan\u00e7ament de l\u00b4associaci\u00f3 americana sense \u00e0nim de lucre <strong>\u201cTogether Strong NPC Foundation\u201d<\/strong>.<\/p>\n<p>Tal com expliquen els investigadors, SAM podria representar una ter\u00e0pia prometedora per al tractament de NPC ja que \u00e9s segura i f\u00e0cil d&#8217;aconseguir, i actualment s&#8217;utilitza per al tractament de malalties psiqui\u00e0triques, neurol\u00f2giques i hep\u00e0tiques, \u00e9s d&#8217;\u00fas oral i presenta bona toler\u00e0ncia. Aix\u00f2 no obstant, es requereixen estudis addicionals per determinar si el tractament de SAM pot funcionar b\u00e9 en conjunci\u00f3 amb altres tractaments aprovats per les autoritats sanit\u00e0ries.<\/p>\n<p>&nbsp;<\/p>\n<p>Leire Goicoechea et al., <strong>S-adenosyl-L-methionine restores brain mitochondrial membrane fluidity and GSH content improving Niemann-Pick type C disease<\/strong>. <a href=\"https:\/\/doi.org\/10.1016\/j.redox.2024.103150\">https:\/\/doi.org\/10.1016\/j.redox.2024.103150<\/a>.<\/p>\n<p style=\"text-align: right;\"><strong>IQAC-CSIC Comunicaci\u00f3<\/strong><\/p>\n","protected":false},"excerpt":{"rendered":"<p>La malaltia de Niemann-Pick tipus C \u00e9s un trastorn hereditari per al qual no hi ha tractament, i que est\u00e0 causat per nivells baixos de les prote\u00efnes NPC1 i NPC2, que regulen el colesterol als lisosomes (org\u00e0nuls de les c\u00e8l\u00b7lules). En conseq\u00fc\u00e8ncia, el cos no pot descompondre apropiadament el colesterol, que s&#8217;acumula a les c\u00e8l\u00b7lules<a class=\"more-link\" href=\"https:\/\/www.iqac.csic.es\/ca\/un-complement-alimentari-te-efectes-beneficiosos-en-la-malaltia-de-niemann-pick-tipus-c\/\">Continue reading <span class=\"screen-reader-text\">\u00abUn complement alimentari t\u00e9 efectes beneficiosos en la malaltia de Niemann-Pick tipus C\u00bb<\/span><\/a><\/p>\n","protected":false},"author":3,"featured_media":4462,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[73],"tags":[],"class_list":["post-8267","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-news","entry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.7 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Un complement alimentari t\u00e9 efectes beneficiosos en la malaltia de Niemann-Pick tipus C | IQAC - Institut de Qu\u00edmica Avan\u00e7ada de Catalunya - CSIC<\/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.iqac.csic.es\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\/\" \/>\n<meta property=\"og:locale\" content=\"ca_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Un complement alimentari t\u00e9 efectes beneficiosos en la malaltia de Niemann-Pick tipus C | IQAC - Institut de Qu\u00edmica Avan\u00e7ada de Catalunya - CSIC\" \/>\n<meta property=\"og:description\" content=\"La malaltia de Niemann-Pick tipus C \u00e9s un trastorn hereditari per al qual no hi ha tractament, i que est\u00e0 causat per nivells baixos de les prote\u00efnes NPC1 i NPC2, que regulen el colesterol als lisosomes (org\u00e0nuls de les c\u00e8l\u00b7lules). En conseq\u00fc\u00e8ncia, el cos no pot descompondre apropiadament el colesterol, que s&#8217;acumula a les c\u00e8l\u00b7lulesContinue reading &quot;Un complement alimentari t\u00e9 efectes beneficiosos en la malaltia de Niemann-Pick tipus C&quot;\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.iqac.csic.es\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\/\" \/>\n<meta property=\"og:site_name\" content=\"IQAC - Institut de Qu\u00edmica Avan\u00e7ada de Catalunya - CSIC\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/iqac.csic.es\/\" \/>\n<meta property=\"article:published_time\" content=\"2024-04-24T11:05:28+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2026-06-16T08:47:24+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.iqac.csic.es\/wp-content\/uploads\/2026\/06\/Imagen.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"640\" \/>\n\t<meta property=\"og:image:height\" content=\"480\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"Ana Sotres\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@IQAC_CSIC\" \/>\n<meta name=\"twitter:site\" content=\"@IQAC_CSIC\" \/>\n<meta name=\"twitter:label1\" content=\"Escrit per\" \/>\n\t<meta name=\"twitter:data1\" content=\"Ana Sotres\" \/>\n\t<meta name=\"twitter:label2\" content=\"Temps estimat de lectura\" \/>\n\t<meta name=\"twitter:data2\" content=\"2 minuts\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/www.iqac.csic.es\\\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/www.iqac.csic.es\\\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\\\/\"},\"author\":{\"name\":\"Ana Sotres\",\"@id\":\"https:\\\/\\\/www.iqac.csic.es\\\/#\\\/schema\\\/person\\\/ac65d00561d3a6d2810128c46771aff7\"},\"headline\":\"Un complement alimentari t\u00e9 efectes beneficiosos en la malaltia de Niemann-Pick tipus C\",\"datePublished\":\"2024-04-24T11:05:28+00:00\",\"dateModified\":\"2026-06-16T08:47:24+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/www.iqac.csic.es\\\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\\\/\"},\"wordCount\":382,\"publisher\":{\"@id\":\"https:\\\/\\\/www.iqac.csic.es\\\/#organization\"},\"image\":{\"@id\":\"https:\\\/\\\/www.iqac.csic.es\\\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\\\/#primaryimage\"},\"thumbnailUrl\":\"https:\\\/\\\/www.iqac.csic.es\\\/wp-content\\\/uploads\\\/2026\\\/06\\\/Imagen.jpg\",\"articleSection\":[\"News\"],\"inLanguage\":\"ca\"},{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/www.iqac.csic.es\\\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\\\/\",\"url\":\"https:\\\/\\\/www.iqac.csic.es\\\/un-complemento-alimenticio-tiene-efectos-beneficiosos-en-la-enfermedad-de-niemann-pick-tipo-c\\\/\",\"name\":\"Un complement alimentari t\u00e9 efectes beneficiosos en la malaltia de Niemann-Pick tipus C | IQAC - 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