{"id":114913,"date":"2024-01-25T11:49:35","date_gmt":"2024-01-25T10:49:35","guid":{"rendered":"https:\/\/ibecbarcelona.eu\/organoids-to-study-ataxia-telangiectasia\/"},"modified":"2024-01-25T11:56:45","modified_gmt":"2024-01-25T10:56:45","slug":"organoids-to-study-ataxia-telangiectasia","status":"publish","type":"post","link":"https:\/\/ibecbarcelona.eu\/ca\/organoides-per-estudiar-ataxia-telangiectasi","title":{"rendered":"Organoides per estudiar l&#8217;at\u00e0xia telangi\u00e8ctasi"},"content":{"rendered":"\n<p><strong>N\u00faria Montserrat Pulido, investigadora principal de l&#8217;IBEC, liderar\u00e0 un projecte per estudiar l&#8217;At\u00e0xia telangi\u00e8ctasi gr\u00e0cies al finan\u00e7ament de l&#8217;AEFAT, l&#8217;associaci\u00f3 espanyola fam\u00edlia at\u00e0xia-telangi\u00e8ctasi. La idea del projecte \u00e9s l&#8217;obtenci\u00f3 de c\u00e8l\u00b7lules amb les mutacions dels pacients, desenvolupar organoides, contemplar la possibilitat d&#8217;assajar processos d&#8217;edici\u00f3 g\u00e8nica per a diferents mutacions i crear una plataforma per testar f\u00e0rmacs.<\/strong><\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" width=\"1024\" height=\"706\" data-src=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2024\/01\/NMontserrat-low-1024x706.jpg\" alt=\"\" class=\"wp-image-114909 lazyload\" data-srcset=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2024\/01\/NMontserrat-low-1024x706.jpg 1024w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2024\/01\/NMontserrat-low-300x207.jpg 300w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2024\/01\/NMontserrat-low-768x529.jpg 768w, https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2024\/01\/NMontserrat-low.jpg 1200w\" data-sizes=\"(max-width: 1024px) 100vw, 1024px\" src=\"data:image\/svg+xml;base64,PHN2ZyB3aWR0aD0iMSIgaGVpZ2h0PSIxIiB4bWxucz0iaHR0cDovL3d3dy53My5vcmcvMjAwMC9zdmciPjwvc3ZnPg==\" style=\"--smush-placeholder-width: 1024px; --smush-placeholder-aspect-ratio: 1024\/706;\" \/><\/figure>\n\n\n\n<p>L&#8217;IBEC s&#8217;uneix a l <a href=\"http:\/\/www.aefat.es\/\">&#8216;Aefat<\/a>, l&#8217;associaci\u00f3 que agrupa les fam\u00edlies afectades per at\u00e0xia telangi\u00e8ctasi a Espanya, en la seva lluita per la investigaci\u00f3 d&#8217;aquesta malaltia rara que afecta almenys 40 nens i joves al pa\u00eds.<strong> <\/strong>Ho fa a trav\u00e9s del projecte titulat &#8220;Modelling Ataxia Telangiectasia pathogenesis and therapeutics using human pluripotent stem cells and genetic engineering&#8221;, liderat per N\u00faria Montserrat Pulido, professora de recerca ICREA i investigadora principal del grup de <a href=\"https:\/\/ibecbarcelona.eu\/es\/ipscs\">Pluripot\u00e8ncia per a la regeneraci\u00f3 d&#8217;\u00f2rgans de l&#8217;<\/a> IBEC.<\/p>\n\n\n\n<p>El projecte de Montserrat \u00e9s un dels dos seleccionats pel Comit\u00e8 Cient\u00edfic de l&#8217;Aefat en la seva segona convocat\u00f2ria internacional de projectes de recerca per buscar una cura o tractament per a aquesta malaltia gen\u00e8tica, neurodegenerativa i multisist\u00e8mica, poc investigada, que encara no t\u00e9 cura ni tractament.<\/p>\n\n\n\n<p>La idea de l&#8217;estudi \u00e9s l&#8217;obtenci\u00f3 de c\u00e8l\u00b7lules iPSc amb les mutacions dels pacients, desenvolupar organoides, contemplar la possibilitat d&#8217;assajar processos d&#8217;edici\u00f3 g\u00e8nica per a diferents mutacions i crear una plataforma per testar f\u00e0rmacs.<\/p>\n\n\n\n<p>Com detalla N\u00faria Montserrat, &#8220;el nostre projecte col\u00b7laboratiu t\u00e9 com a objectiu establir nous models cel\u00b7lulars per comprendre l&#8217;at\u00e0xia telangi\u00e8ctasi, que es produeix per mutacions en el gen ATM. Usarem enginyeria gen\u00e8tica i enginyeria cel\u00b7lular per, d&#8217;una banda, generar c\u00e8l\u00b7lules que presentin mutacions causals de la malaltia i per l&#8217;altra banda, generar els diferents tipus de c\u00e8l\u00b7lules i teixits que estan afectats en aquests pacients. Per a aquest segon objectiu, establirem cultius que anomenem organoides, que s\u00f3n sistemes cel\u00b7lulars que podem generar al laboratori que s&#8217;assemblen a l&#8217;\u00f2rgan que volem estudiar&#8221;.<\/p>\n\n\n\n<p>L&#8217;objectiu, com assenyala la bi\u00f2loga i investigadora, &#8220;\u00e9s generar una plataforma cel\u00b7lular per estudiar processos que s\u00f3n importants durant l&#8217;at\u00e0xia telangi\u00e8ctasi, i el que \u00e9s tamb\u00e9 molt important, vam poder explotar aquesta plataforma cel\u00b7lular per testar vectors i compostos terap\u00e8utics, que ens permetin corregir els defectes que es produeixen a les c\u00e8l\u00b7lules i mini\u00f2rgans creats al laboratori a causa de la pres\u00e8ncia de mutacions al gen ATM&#8221;.\u00a0 A m\u00e9s. &#8220;vam\u00a0 incorporar la participaci\u00f3 d&#8217;infraestructures com la Plataforma de Biomodelos i Biobancs de l&#8217;Institut de Salut Carles III, a trav\u00e9s de la qual assegurarem la incorporaci\u00f3 i cust\u00f2dia de les mostres biol\u00f2giques del projecte. I comptem amb la col\u00b7laboraci\u00f3 de Jordi Surrall\u00e9s, doctor en gen\u00e8tica i director de l&#8217;Institut de Recerca de l&#8217;Hospital Sant Pau&#8221;.<\/p>\n\n\n\n<p>Per a una major efici\u00e8ncia d&#8217;aquest projecte, Aefat ha posat en contacte l&#8217;equip amb investigadors internacionals que estan treballant en el mateix camp i amb altres professionals que ja han participat en projectes finan\u00e7ats per l&#8217;associaci\u00f3, com Marc G\u00fcell (Universitat Pompeu Fabra).<\/p>\n\n\n\n<p>Els nous projectes estan valorats en 150.000 euros cadascun, amb una durada de 18-24 mesos. L&#8217;esfor\u00e7 d&#8217;inversi\u00f3 realitzat per Aefat s&#8217;ha vist recolzat amb les aportacions d&#8217;associacions d&#8217;altres pa\u00efsos, dues en concret de les nou amb les quals comparteixen l<a href=\"https:\/\/cureat.org\/\">&#8216;A-T Global Alliance<\/a>, l&#8217;alian\u00e7a creada el 2020 per avan\u00e7ar en la investigaci\u00f3 i conscienciaci\u00f3 a escala internacional. L&#8217;associaci\u00f3 anglesa <a href=\"https:\/\/actionforat.org\/\">Action for A-T<\/a> i l&#8217;australiana <a href=\"https:\/\/brashat.org.au\/\">BrAshA-T<\/a> col\u00b7laboren en el finan\u00e7ament d&#8217;aquests dos nous projectes llan\u00e7ats des d&#8217;Espanya.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>N\u00faria Montserrat Pulido, investigadora principal de l&#8217;IBEC, liderar\u00e0 un projecte per estudiar l&#8217;At\u00e0xia telangi\u00e8ctasi gr\u00e0cies al finan\u00e7ament de l&#8217;AEFAT, l&#8217;associaci\u00f3 espanyola fam\u00edlia at\u00e0xia-telangi\u00e8ctasi. La idea del projecte \u00e9s l&#8217;obtenci\u00f3 de c\u00e8l\u00b7lules amb les mutacions dels pacients, desenvolupar organoides, contemplar la possibilitat d&#8217;assajar processos d&#8217;edici\u00f3 g\u00e8nica per a diferents mutacions i crear una plataforma per testar f\u00e0rmacs.<\/p>\n","protected":false},"author":37,"featured_media":114910,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[98],"tags":[1123,1780],"post_formats":[],"class_list":["post-114913","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-noticies-institucionals","tag-nuria-montserrat-ca-2","tag-pluripotency-for-organ-regeneration-ca"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.4 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Organoides per estudiar l&#039;at\u00e0xia telangi\u00e8ctasi - 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\/organoides-per-estudiar-ataxia-telangiectasi\/\" \/>\n<meta property=\"og:locale\" content=\"ca_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Organoides per estudiar l&#039;at\u00e0xia telangi\u00e8ctasi - Institute for Bioengineering of Catalonia\" \/>\n<meta property=\"og:description\" content=\"N\u00faria Montserrat Pulido, investigadora principal de l&#039;IBEC, liderar\u00e0 un projecte per estudiar l&#039;At\u00e0xia telangi\u00e8ctasi gr\u00e0cies al finan\u00e7ament de l&#039;AEFAT, l&#039;associaci\u00f3 espanyola fam\u00edlia at\u00e0xia-telangi\u00e8ctasi. La idea del projecte \u00e9s l&#039;obtenci\u00f3 de c\u00e8l\u00b7lules amb les mutacions dels pacients, desenvolupar organoides, contemplar la possibilitat d&#039;assajar processos d&#039;edici\u00f3 g\u00e8nica per a diferents mutacions i crear una plataforma per testar f\u00e0rmacs.\" \/>\n<meta property=\"og:url\" content=\"https:\/\/ibecbarcelona.eu\/ca\/organoides-per-estudiar-ataxia-telangiectasi\/\" \/>\n<meta property=\"og:site_name\" content=\"Institute for Bioengineering of Catalonia\" \/>\n<meta property=\"article:published_time\" content=\"2024-01-25T10:49:35+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2024-01-25T10:56:45+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/ibecbarcelona.eu\/wp-content\/uploads\/2024\/01\/NMontserrat-low.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"1200\" \/>\n\t<meta property=\"og:image:height\" content=\"827\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"Sarah Moreira\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Escrit per\" \/>\n\t<meta name=\"twitter:data1\" content=\"Sarah Moreira\" \/>\n\t<meta name=\"twitter:label2\" content=\"Temps estimat de lectura\" \/>\n\t<meta name=\"twitter:data2\" content=\"3 minuts\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/ibecbarcelona.eu\\\/ca\\\/organoides-per-estudiar-ataxia-telangiectasi\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/ibecbarcelona.eu\\\/ca\\\/organoides-per-estudiar-ataxia-telangiectasi\\\/\"},\"author\":{\"name\":\"Sarah Moreira\",\"@id\":\"https:\\\/\\\/ibecbarcelona.eu\\\/#\\\/schema\\\/person\\\/d3a158b8d448000777023ca1e3d71d34\"},\"headline\":\"Organoides per estudiar l&#8217;at\u00e0xia telangi\u00e8ctasi\",\"datePublished\":\"2024-01-25T10:49:35+00:00\",\"dateModified\":\"2024-01-25T10:56:45+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/ibecbarcelona.eu\\\/ca\\\/organoides-per-estudiar-ataxia-telangiectasi\\\/\"},\"wordCount\":622,\"publisher\":{\"@id\":\"https:\\\/\\\/ibecbarcelona.eu\\\/#organization\"},\"image\":{\"@id\":\"https:\\\/\\\/ibecbarcelona.eu\\\/ca\\\/organoides-per-estudiar-ataxia-telangiectasi\\\/#primaryimage\"},\"thumbnailUrl\":\"https:\\\/\\\/ibecbarcelona.eu\\\/wp-content\\\/uploads\\\/2024\\\/01\\\/NMontserrat-low.jpg\",\"keywords\":[\"Nuria Montserrat\",\"Pluripotency for organ regeneration\"],\"articleSection\":[\"Not\u00edcies institucionals\"],\"inLanguage\":\"ca\"},{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/ibecbarcelona.eu\\\/ca\\\/organoides-per-estudiar-ataxia-telangiectasi\\\/\",\"url\":\"https:\\\/\\\/ibecbarcelona.eu\\\/ca\\\/organoides-per-estudiar-ataxia-telangiectasi\\\/\",\"name\":\"Organoides per estudiar l'at\u00e0xia telangi\u00e8ctasi - 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