{"id":941,"date":"2024-09-29T16:57:59","date_gmt":"2024-09-29T14:57:59","guid":{"rendered":"https:\/\/rvh-synergie.org\/actualites\/les-traitements-qui-maintiennent-la-sante-des-synapses-peuvent-aider-a-prevenir-et-a-attenuer-les-symptomes-de-la-maladie-a-prions\/"},"modified":"2024-09-29T16:58:08","modified_gmt":"2024-09-29T14:58:08","slug":"les-traitements-qui-maintiennent-la-sante-des-synapses-peuvent-aider-a-prevenir-et-a-attenuer-les-symptomes-de-la-maladie-a-prions","status":"publish","type":"post","link":"https:\/\/rvh-synergie.org\/actualites\/les-traitements-qui-maintiennent-la-sante-des-synapses-peuvent-aider-a-prevenir-et-a-attenuer-les-symptomes-de-la-maladie-a-prions\/","title":{"rendered":"Les traitements qui maintiennent la sant\u00e9 des synapses peuvent aider \u00e0 pr\u00e9venir et \u00e0 att\u00e9nuer les sympt\u00f4mes de la maladie \u00e0 prions"},"content":{"rendered":"<div>\n<div class=\"article-gallery lightGallery\">\n<div data-thumb=\"https:\/\/scx1.b-cdn.net\/csz\/news\/tmb\/2024\/new-study-suggest-trea.jpg\" data-src=\"https:\/\/scx2.b-cdn.net\/gfx\/news\/hires\/2024\/new-study-suggest-trea.jpg\" data-sub-html=\"Differentiation of human iPSCs to pyramidal cortical neurons via dual SMAD inhibition. Credit: &lt;i&gt;Stem Cell Reports&lt;\/i&gt; (2024). DOI: 10.1016\/j.stemcr.2024.08.010\">\n<figure class=\"article-img\">\n<\/figure><\/div>\n<\/div>\n<p>La maladie g\u00e9n\u00e9tique \u00e0 prions se manifeste g\u00e9n\u00e9ralement par des difficult\u00e9s cognitives, un mauvais contr\u00f4le musculaire et des mouvements saccad\u00e9s brusques de groupes musculaires et\/ou de membres entiers. Les trois principaux ph\u00e9notypes de la maladie g\u00e9n\u00e9tique \u00e0 prions sont la maladie g\u00e9n\u00e9tique de Creutzfeldt-Jakob (MCJ), l&rsquo;insomnie familiale mortelle (FFI) et le syndrome de Gerstmann-Str\u00e4ussler-Scheinker (GSS). La cause la plus fr\u00e9quente des maladies h\u00e9r\u00e9ditaires \u00e0 prions est la mutation E200K de la prot\u00e9ine prion (PrP). On pense souvent que cette mutation provoque une maladie en rendant la PrP plus susceptible de se replier mal en une forme pathog\u00e8ne (PrP<sup>Sc<\/sup>).<\/p>\n<p>Cependant, de nouvelles recherches men\u00e9es par la Chobanian &#038; Avedisian School of Medicine de l&rsquo;Universit\u00e9 de Boston et le Boston Medical Center (BMC) ont d\u00e9couvert que l&rsquo;architecture des sites de contact neurone \u00e0 neurone, appel\u00e9s synapses, est alt\u00e9r\u00e9e dans les neurones exprimant cette PrP mutante en l&rsquo;absence de PrP<sup>Sc<\/sup>. Ceci sugg\u00e8re qu&rsquo;une perte ou une modification de la fonction de la PrP pourrait contribuer au ph\u00e9notype de la maladie.<\/p>\n<p>L&rsquo;\u00e9tude est publi\u00e9e dans la revue <i>Rapports sur les cellules souches<\/i>.<\/p>\n<p>\u00ab\u00a0Nos r\u00e9sultats sugg\u00e8rent qu&rsquo;il pourrait y avoir des anomalies d\u00e9tectables dans les neurones bien avant l&rsquo;apparition des principaux sympt\u00f4mes des maladies h\u00e9r\u00e9ditaires \u00e0 prions\u00a0\u00bb, a expliqu\u00e9 l&rsquo;auteur co-correspondant David A. Harris, MD, Ph.D., professeur Edgar Minas Housepian et pr\u00e9sident du d\u00e9partement de biochimie et de biologie cellulaire de l&rsquo;\u00e9cole.<\/p>\n<p>Harris et ses coll\u00e8gues ont constitu\u00e9 une vaste biblioth\u00e8que de cellules souches pluripotentes induites (CSPi) (cellules sanguines qui ont \u00e9t\u00e9 reprogramm\u00e9es dans un \u00e9tat pluripotent de type embryonnaire qui permet le d\u00e9veloppement d&rsquo;une source illimit\u00e9e de tout type de cellule humaine n\u00e9cessaire \u00e0 des fins th\u00e9rapeutiques). issus d&rsquo;une famille h\u00e9bergeant cette mutation, et les a diff\u00e9renci\u00e9s en neurones.<\/p>\n<p>Ils ont ensuite compar\u00e9 les neurones porteurs de mutations aux neurones non porteurs. Ils ont \u00e9galement utilis\u00e9 la technologie CRISPR\/Cas9 pour corriger la mutation dans deux lign\u00e9es, afin de pouvoir effectuer ces comparaisons entre des neurones ayant des ant\u00e9c\u00e9dents g\u00e9n\u00e9tiques identiques en dehors de la mutation d\u2019int\u00e9r\u00eat.<\/p>\n<p>Selon les chercheurs, l\u2019utilisation de la technologie iPSC constitue un pas de plus vers la m\u00e9decine personnalis\u00e9e. \u00ab\u00a0Notre \u00e9tude utilise la plus grande collection d&rsquo;iPSC provenant d&rsquo;une famille h\u00e9bergeant une maladie \u00e0 prions h\u00e9r\u00e9ditaire \u00e0 notre connaissance. Les neurones d\u00e9riv\u00e9s d&rsquo;iPSC peuvent fournir d&rsquo;importantes informations m\u00e9canistiques sur la pathogen\u00e8se des maladies g\u00e9n\u00e9tiques \u00e0 prions et peuvent offrir une plate-forme puissante pour tester des th\u00e9rapies candidates. \u00ab\u00a0, a d\u00e9clar\u00e9 l&rsquo;auteur co-correspondant Gustavo Mostoslavsky, MD, Ph.D., professeur de m\u00e9decine et de microbiologie \u00e0 l&rsquo;\u00e9cole et codirecteur du BU et du BMC Center for Regenerative Medicine.<\/p>\n<p>Les chercheurs pensent que cette \u00e9tude fait progresser la compr\u00e9hension de ce groupe rare mais d\u00e9vastateur de troubles neurod\u00e9g\u00e9n\u00e9ratifs qui endommagent les connexions entre les cellules nerveuses du cerveau, et fournit un guide sur les traitements qui pourraient \u00eatre les plus efficaces pour combattre les sympt\u00f4mes de ces troubles.<\/p>\n<p>\u00ab\u00a0Des approches th\u00e9rapeutiques similaires pourraient \u00e9galement s&rsquo;appliquer \u00e0 la maladie d&rsquo;Alzheimer et \u00e0 d&rsquo;autres maladies neurod\u00e9g\u00e9n\u00e9ratives, dont certains cas sont h\u00e9r\u00e9ditaires\u00a0\u00bb, a d\u00e9clar\u00e9 Harris.<\/p>\n<p>                                        <!-- print only --><\/p><\/div>\n","protected":false},"excerpt":{"rendered":"<p>La maladie g\u00e9n\u00e9tique \u00e0 prions se manifeste g\u00e9n\u00e9ralement par des difficult\u00e9s cognitives, un mauvais contr\u00f4le<\/p>\n","protected":false},"author":1,"featured_media":942,"comment_status":"closed","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[86],"tags":[264,1211,16,49,1209,248,92,538,1212,193,212,42,1210,390],"class_list":["post-941","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-les-maladies","tag-aider","tag-attenuer","tag-des","tag-les","tag-maintiennent","tag-maladie","tag-peuvent","tag-prevenir","tag-prions","tag-qui","tag-sante","tag-symptomes","tag-synapses","tag-traitements","generate-columns","tablet-grid-50","mobile-grid-100","grid-parent","grid-50","resize-featured-image"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v23.4 - 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