{"id":363895,"date":"2024-01-28T21:48:32","date_gmt":"2024-01-28T21:48:32","guid":{"rendered":"https:\/\/afrikmag.com\/var\/www\/vhosts\/afrikmag.compublic_html\/?p=363895"},"modified":"2024-01-28T21:48:32","modified_gmt":"2024-01-28T21:48:32","slug":"un-jeune-garcon-ne-sourd-entend-pour-la-premiere-fois-grace-a-une-therapie-genique-revolutionnaire","status":"publish","type":"post","link":"https:\/\/afrikmag.com\/var\/www\/vhosts\/afrikmag.compublic_html\/un-jeune-garcon-ne-sourd-entend-pour-la-premiere-fois-grace-a-une-therapie-genique-revolutionnaire\/","title":{"rendered":"Un jeune gar\u00e7on n\u00e9 sourd entend pour la premi\u00e8re fois gr\u00e2ce \u00e0 une th\u00e9rapie g\u00e9nique r\u00e9volutionnaire"},"content":{"rendered":"<p><span>Un traitement r\u00e9volutionnaire a permis \u00e0 six enfants sourds, \u00e2g\u00e9s de 1 \u00e0 11 ans, de faire l&rsquo;exp\u00e9rience de l&rsquo;audition pour la premi\u00e8re fois.\u00a0Les enfants, tous n\u00e9s avec une mutation g\u00e9n\u00e9tique emp\u00eachant la\u00a0<\/span><span>production<\/span><span>\u00a0d&rsquo;une prot\u00e9ine auditive essentielle, ont particip\u00e9 \u00e0 des groupes exp\u00e9rimentaux en Chine et aux \u00c9tats-Unis.<\/span><\/p>\n<p><span>Les scientifiques ont administr\u00e9 une version du g\u00e8ne, connue sous le nom d&rsquo;otoferline (OTOF), dans l&rsquo;oreille interne, incitant les cellules \u00e0 produire la prot\u00e9ine pr\u00e9c\u00e9demment manquante, selon le\u00a0<\/span><span>Daily Mail<\/span><span>.\u00a0Apr\u00e8s 26 semaines de traitement, les niveaux auditifs des enfants se sont am\u00e9lior\u00e9s jusqu&rsquo;\u00e0 atteindre 70 pour cent\u00a0<\/span><span>de la normale<\/span><span>.<\/span><\/p>\n<p><span>Des progr\u00e8s notables ont \u00e9t\u00e9 observ\u00e9s d\u00e8s six semaines de traitement.\u00a0<\/span><span>Des vid\u00e9os montrent un enfant d&rsquo;un an r\u00e9pondant \u00e0 son nom pour la premi\u00e8re fois, et une autre fille articulant des mots comme p\u00e8re, m\u00e8re, grand-m\u00e8re, s\u0153ur et \u00ab Je t&rsquo;aime \u00bb, ce qu&rsquo;elle ne pouvait pas faire auparavant.<\/span><\/p>\n<p><span>Aissam Dam, 11 ans, a entendu pour la premi\u00e8re fois cette semaine \u00e0 l&rsquo;h\u00f4pital pour enfants de Philadelphie (CHOP) apr\u00e8s avoir re\u00e7u un traitement \u2013 \u200b\u200bce qui constitue une premi\u00e8re aux \u00c9tats-Unis.<\/span><\/p>\n<p><span>Selon Zheng-Yi Chen, DPhil,\u00a0<\/span><span>professeur<\/span><span>\u00a0\u00e0 la Harvard Medical School et auteur de l&rsquo;\u00e9tude sur les exp\u00e9riences chinoises, une d\u00e9ficience auditive non trait\u00e9e chez les enfants peut entra\u00eener un d\u00e9veloppement c\u00e9r\u00e9bral anormal.<\/span><\/p>\n<p><span>La th\u00e9rapie g\u00e9nique, d\u00e9j\u00e0 approuv\u00e9e pour des pathologies telles que la dr\u00e9panocytose et l&rsquo;h\u00e9mophilie s\u00e9v\u00e8re, est d\u00e9sormais explor\u00e9e pour la surdit\u00e9 h\u00e9r\u00e9ditaire.\u00a0<\/span><span>Dans le monde<\/span><span>\u00a0, environ 34 millions d&rsquo;enfants souffrent de surdit\u00e9 ou de perte auditive, les g\u00e8nes repr\u00e9sentant jusqu&rsquo;\u00e0 60 pour cent des cas.<\/span><\/p>\n<p><span>Dam, qui est n\u00e9e \u00ab profond\u00e9ment sourde \u00bb, et cinq enfants en Chine partagent une anomalie tr\u00e8s rare dans leur g\u00e8ne OTOF, entra\u00eenant un g\u00e8ne d\u00e9fectueux qui entrave la production d&rsquo;otoferline.\u00a0L&rsquo;otoferline est une prot\u00e9ine cruciale n\u00e9cessaire aux \u00ab cellules cili\u00e9es \u00bb de l&rsquo;oreille interne, charg\u00e9es de convertir les vibrations sonores en signaux chimiques envoy\u00e9s au\u00a0<\/span><span>cerveau<\/span><span>\u00a0.<\/span><\/p>\n<p><span>Les anomalies du g\u00e8ne Otoferlin sont rares et constituent un \u00e0 huit pour cent des cas de perte auditive \u00e0 la naissance.<\/span><\/p>\n<p><span>En octobre, Dam a subi une intervention chirurgicale pour soigner sa surdit\u00e9.\u00a0La proc\u00e9dure consistait \u00e0 soulever partiellement son tympan et \u00e0 injecter un virus inoffensif, modifi\u00e9 pour transporter des copies fonctionnelles du g\u00e8ne de l&rsquo;otoferline, dans le liquide interne de sa cochl\u00e9e.<\/span><\/p>\n<p><span>Par cons\u00e9quent, les cellules cili\u00e9es de son oreille interne ont commenc\u00e9 \u00e0 produire la prot\u00e9ine qui leur manquait auparavant, leur permettant ainsi de fonctionner correctement.<\/span><\/p>\n<p><span>Pr\u00e8s de quatre mois apr\u00e8s le traitement d&rsquo;une oreille, l&rsquo;audition de Dam s&rsquo;est consid\u00e9rablement am\u00e9lior\u00e9e, ne laissant subsister qu&rsquo;une perte auditive l\u00e9g\u00e8re \u00e0 mod\u00e9r\u00e9e.\u00a0Selon un communiqu\u00e9 de l\u2019H\u00f4pital pour enfants de Philadelphie (CHOP), Dam \u00ab entend litt\u00e9ralement un son pour la premi\u00e8re fois de sa vie \u00bb.<\/span><\/p>\n<p><span>Le Dr John A. Germiller du CHOP a d\u00e9clar\u00e9 que la th\u00e9rapie g\u00e9nique pour la perte auditive, un objectif poursuivi depuis plus de deux d\u00e9cennies, est d\u00e9sormais devenue une r\u00e9alit\u00e9.\u00a0Le succ\u00e8s de la correction d&rsquo;une anomalie g\u00e9n\u00e9tique rare dans le cas de Dam pourrait ouvrir la voie \u00e0 de futures applications ciblant plus de 150 autres g\u00e8nes responsables de la perte auditive chez l&rsquo;enfant.<\/span><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Un traitement r\u00e9volutionnaire a permis \u00e0 six enfants sourds, \u00e2g\u00e9s de 1 \u00e0 11 ans, de faire l&rsquo;exp\u00e9rience de l&rsquo;audition pour la premi\u00e8re fois.\u00a0Les enfants, tous n\u00e9s avec une mutation g\u00e9n\u00e9tique emp\u00eachant la\u00a0production\u00a0d&rsquo;une prot\u00e9ine auditive essentielle, ont particip\u00e9 \u00e0 des groupes exp\u00e9rimentaux en Chine et aux \u00c9tats-Unis. Les scientifiques ont administr\u00e9 une version du g\u00e8ne, &hellip;<\/p>\n","protected":false},"author":45,"featured_media":363896,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"image","meta":{"footnotes":""},"categories":[189118,7020,46],"tags":[199509,199507,199508],"class_list":["post-363895","post","type-post","status-publish","format-image","has-post-thumbnail","hentry","category-a-la-une","category-societe","category-usa","tag-aissam-dam","tag-garcon-ne-sourd","tag-mutation-genetique","post_format-post-format-image"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.5 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Un jeune gar\u00e7on n\u00e9 sourd entend pour la premi\u00e8re fois gr\u00e2ce \u00e0 une th\u00e9rapie g\u00e9nique r\u00e9volutionnaire - AfrikMag<\/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:\/\/afrikmag.com\/var\/www\/vhosts\/afrikmag.compublic_html\/un-jeune-garcon-ne-sourd-entend-pour-la-premiere-fois-grace-a-une-therapie-genique-revolutionnaire\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Un jeune gar\u00e7on n\u00e9 sourd entend pour la premi\u00e8re fois gr\u00e2ce \u00e0 une th\u00e9rapie g\u00e9nique r\u00e9volutionnaire - AfrikMag\" \/>\n<meta property=\"og:description\" content=\"Un traitement r\u00e9volutionnaire a permis \u00e0 six enfants sourds, \u00e2g\u00e9s de 1 \u00e0 11 ans, de faire l&rsquo;exp\u00e9rience de l&rsquo;audition pour la premi\u00e8re fois.\u00a0Les enfants, tous n\u00e9s avec une mutation g\u00e9n\u00e9tique emp\u00eachant la\u00a0production\u00a0d&rsquo;une prot\u00e9ine auditive essentielle, ont particip\u00e9 \u00e0 des groupes exp\u00e9rimentaux en Chine et aux \u00c9tats-Unis. 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