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text_font_size_last_edited=\u00a0\u00bbon|phone\u00a0\u00bb header_2_font_size_tablet=\u00a0\u00bb32px\u00a0\u00bb header_2_font_size_phone=\u00a0\u00bb22px\u00a0\u00bb header_2_font_size_last_edited=\u00a0\u00bbon|desktop\u00a0\u00bb locked=\u00a0\u00bboff\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb]<\/p>\n<h1 style=\"font-weight: 400; text-align: center;\"><span style=\"font-size: large; color: #008080;\"><\/span><\/h1>\n<h1 style=\"font-weight: 400; text-align: center;\"><span style=\"font-size: large; color: #008080;\"><\/span><\/h1>\n<h1 style=\"font-weight: 400; text-align: center;\"><span style=\"font-size: large; color: #008080;\">Vous trouverez ci-dessous une liste d&rsquo;initiatives de recherche que nous avons fi\u00e8rement soutenues. Ces projets sont au c\u0153ur de notre mission, qui consiste \u00e0 \u00e9tablir une collaboration avec des chercheurs et des cliniciens d\u00e9vou\u00e9s, afin d&rsquo;\u00e9lucider la biologie de la maladie <em>li\u00e9e au CLCN4<\/em> et d&rsquo;am\u00e9liorer la qualit\u00e9 de vie des personnes qui en sont atteintes. <\/span><\/h1>\n<p>[\/et_pb_text][\/et_pb_column][et_pb_column type=\u00a0\u00bb1_2&Prime; _builder_version=\u00a0\u00bb4.16&Prime; custom_padding=\u00a0\u00bb|||\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb custom_padding__hover=\u00a0\u00bb|||\u00a0\u00bb][et_pb_image src=\u00a0\u00bbhttps:\/\/cureclcn4.org\/wp-content\/uploads\/2023\/10\/research_funded_proj-1.png\u00a0\u00bb title_text=\u00a0\u00bbprojet_de_recherche_financ\u00e9\u00a0\u00bb _builder_version=\u00a0\u00bb4.23.1&Prime; _module_preset=\u00a0\u00bbdefault\u00a0\u00bb width=\u00a0\u00bb69%\u00a0\u00bb module_alignment=\u00a0\u00bbcenter\u00a0\u00bb custom_margin=\u00a0\u00bb0px||0px||false|false\u00a0\u00bb custom_padding=\u00a0\u00bb0px||0px||false|false\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][\/et_pb_image][\/et_pb_column][\/et_pb_row][et_pb_row custom_padding_last_edited=\u00a0\u00bbon|phone\u00a0\u00bb _builder_version=\u00a0\u00bb4.16&Prime; background_color=\u00a0\u00bb#ffffff\u00a0\u00bb max_width=\u00a0\u00bb1200px\u00a0\u00bb custom_padding=\u00a0\u00bb40px|80px|27px|80px|false|true\u00a0\u00bb custom_padding_tablet=\u00a0\u00bb\u00a0\u00bb custom_padding_phone=\u00a0\u00bb|30px||30px||true\u00a0\u00bb box_shadow_style=\u00a0\u00bbpreset3&Prime; box_shadow_vertical=\u00a0\u00bb40px\u00a0\u00bb box_shadow_blur=\u00a0\u00bb100px\u00a0\u00bb box_shadow_spread=\u00a0\u00bb-30px\u00a0\u00bb box_shadow_color=\u00a0\u00bbrgba(0,0,0,0.22)\u00a0\u00bb use_custom_width=\u00a0\u00bbon\u00a0\u00bb custom_width_px=\u00a0\u00bb1200px\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][et_pb_column type=\u00a0\u00bb4_4&Prime; _builder_version=\u00a0\u00bb4.16&Prime; custom_padding=\u00a0\u00bb|||\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb custom_padding__hover=\u00a0\u00bb|||\u00a0\u00bb][et_pb_text _builder_version=\u00a0\u00bb4.22.2&Prime; text_font=\u00a0\u00bbFaustina||||||||\u00a0\u00bb text_font_size=\u00a0\u00bb24px\u00a0\u00bb text_line_height=\u00a0\u00bb2em\u00a0\u00bb link_font=\u00a0\u00bb|700|||||||\u00a0\u00bb link_text_color=\u00a0\u00bb#26c699&Prime; header_font=\u00a0\u00bb||||||||\u00a0\u00bb header_2_font=\u00a0\u00bbFaustina|600|||||||\u00a0\u00bb header_2_text_color=\u00a0\u00bb#0e7162&Prime; header_2_font_size=\u00a0\u00bb30px\u00a0\u00bb header_2_line_height=\u00a0\u00bb1.4em\u00a0\u00bb text_orientation=\u00a0\u00bbcenter\u00a0\u00bb max_width=\u00a0\u00bb850px\u00a0\u00bb module_alignment=\u00a0\u00bbcenter\u00a0\u00bb text_font_size_tablet=\u00a0\u00bb\u00a0\u00bb text_font_size_phone=\u00a0\u00bb17px\u00a0\u00bb text_font_size_last_edited=\u00a0\u00bbon|phone\u00a0\u00bb header_2_font_size_tablet=\u00a0\u00bb32px\u00a0\u00bb header_2_font_size_phone=\u00a0\u00bb22px\u00a0\u00bb header_2_font_size_last_edited=\u00a0\u00bbon|desktop\u00a0\u00bb locked=\u00a0\u00bboff\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb]<\/p>\n<h2>Ensemble, nous progressons pas \u00e0 pas<\/h2>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][et_pb_row custom_padding_last_edited=\u00a0\u00bbon|phone\u00a0\u00bb _builder_version=\u00a0\u00bb4.16&Prime; background_color=\u00a0\u00bb#ffffff\u00a0\u00bb max_width=\u00a0\u00bb1200px\u00a0\u00bb custom_padding=\u00a0\u00bb29px|80px|28px|80px|false|false\u00a0\u00bb custom_padding_tablet=\u00a0\u00bb|80px||80px|false|true\u00a0\u00bb custom_padding_phone=\u00a0\u00bb40px|30px||30px||true\u00a0\u00bb box_shadow_style=\u00a0\u00bbpreset3&Prime; box_shadow_vertical=\u00a0\u00bb40px\u00a0\u00bb box_shadow_blur=\u00a0\u00bb100px\u00a0\u00bb box_shadow_spread=\u00a0\u00bb-30px\u00a0\u00bb box_shadow_color=\u00a0\u00bbrgba(0,0,0,0.22)\u00a0\u00bb use_custom_width=\u00a0\u00bbon\u00a0\u00bb custom_width_px=\u00a0\u00bb1200px\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][et_pb_column type=\u00a0\u00bb4_4&Prime; _builder_version=\u00a0\u00bb4.16&Prime; custom_padding=\u00a0\u00bb|||\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb custom_padding__hover=\u00a0\u00bb|||\u00a0\u00bb][et_pb_blurb title=\u00a0\u00bbProgramme pilote de subventions Cure clcn4 2023 &#8211; annonce des laur\u00e9ats en MAI 2024&Prime; content_max_width=\u00a0\u00bb800px\u00a0\u00bb _builder_version=\u00a0\u00bb4.23.1&Prime; header_font=\u00a0\u00bb|700||on|||||\u00a0\u00bb header_font_size=\u00a0\u00bb18px\u00a0\u00bb header_line_height=\u00a0\u00bb2em\u00a0\u00bb body_font_size=\u00a0\u00bb17px\u00a0\u00bb body_line_height=\u00a0\u00bb2em\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb]<\/p>\n<p style=\"text-align: justify;\">Les candidatures pour le programme de subvention pilote Cure CLCN4 2023 sont ouvertes (date limite 15 d\u00e9cembre 2023) ! La subvention fournira un total de 150 000 \u00a3 pour 2 ou 3 projets de recherche ax\u00e9s sur la condition <em>li\u00e9e au CLCN4<\/em>(50 000 \u00a3-75 000 \u00a3\/chacun). Les d\u00e9tails des projets r\u00e9compens\u00e9s seront annonc\u00e9s ici au d\u00e9but du mois de mai. Pour plus de d\u00e9tails sur la subvention, veuillez cliquer <a href=\"https:\/\/cureclcn4.org\/cure-clcn4-pilot-grant-program-2023\/\">ici<\/a>.     <strong><\/strong><\/p>\n<p>[\/et_pb_blurb][\/et_pb_column][\/et_pb_row][et_pb_row custom_padding_last_edited=\u00a0\u00bbon|phone\u00a0\u00bb _builder_version=\u00a0\u00bb4.16&Prime; background_color=\u00a0\u00bb#ffffff\u00a0\u00bb max_width=\u00a0\u00bb1200px\u00a0\u00bb custom_padding=\u00a0\u00bb29px|80px|28px|80px|false|false\u00a0\u00bb custom_padding_tablet=\u00a0\u00bb|80px||80px|false|true\u00a0\u00bb custom_padding_phone=\u00a0\u00bb40px|30px||30px||true\u00a0\u00bb box_shadow_style=\u00a0\u00bbpreset3&Prime; box_shadow_vertical=\u00a0\u00bb40px\u00a0\u00bb box_shadow_blur=\u00a0\u00bb100px\u00a0\u00bb box_shadow_spread=\u00a0\u00bb-30px\u00a0\u00bb box_shadow_color=\u00a0\u00bbrgba(0,0,0,0.22)\u00a0\u00bb use_custom_width=\u00a0\u00bbon\u00a0\u00bb custom_width_px=\u00a0\u00bb1200px\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][et_pb_column type=\u00a0\u00bb4_4&Prime; _builder_version=\u00a0\u00bb4.16&Prime; custom_padding=\u00a0\u00bb|||\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb custom_padding__hover=\u00a0\u00bb|||\u00a0\u00bb][et_pb_blurb title=\u00a0\u00bb%912023 &#8211; present%93 &#8211; G\u00e9n\u00e9ration d&#039;anticorps anti-CIC-4 pour la recherche fondamentale\u00a0\u00bb content_max_width=\u00a0\u00bb800px\u00a0\u00bb _builder_version=\u00a0\u00bb4.23.1&Prime; header_font=\u00a0\u00bb|700||on|||||\u00a0\u00bb header_font_size=\u00a0\u00bb18px\u00a0\u00bb header_line_height=\u00a0\u00bb2em\u00a0\u00bb body_font_size=\u00a0\u00bb17px\u00a0\u00bb body_line_height=\u00a0\u00bb2em\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb]<\/p>\n<p style=\"text-align: justify;\"><strong>Raison d&rsquo;\u00eatre :<\/strong> Les anticorps sont des outils essentiels de la recherche fondamentale, car ils permettent la d\u00e9tection sp\u00e9cifique de prot\u00e9ines d&rsquo;int\u00e9r\u00eat. Cette capacit\u00e9 de d\u00e9tection est essentielle pour comprendre les niveaux d&rsquo;expression des prot\u00e9ines, leurs modifications et leur localisation dans les cellules ou les tissus. En utilisant des anticorps, les chercheurs peuvent d\u00e9terminer o\u00f9 et quand une prot\u00e9ine est exprim\u00e9e, observer comment son expression change dans diff\u00e9rentes conditions et obtenir des informations sur son r\u00f4le dans divers processus cellulaires. Il n&rsquo;existe actuellement aucun anticorps valid\u00e9 de mani\u00e8re ind\u00e9pendante et largement disponible pour la d\u00e9tection de CIC-4, la prot\u00e9ine cod\u00e9e par le g\u00e8ne <em>CLCN4<\/em>, ce qui constitue un obstacle pour la recherche fondamentale.   <\/p>\n<p style=\"text-align: justify;\"><strong>Objectif :<\/strong> L&rsquo;objectif de ce projet est de d\u00e9velopper un ensemble d&rsquo;anticorps fonctionnels pour la d\u00e9tection sp\u00e9cifique de la prot\u00e9ine CIC-4. Des projets de g\u00e9n\u00e9ration d&rsquo;un anticorps polyclonal et monoclonal sont en cours (plus de d\u00e9tails \u00e0 venir !). <\/p>\n<p style=\"text-align: justify;\"><strong>R\u00e9sultats (en cours)<\/strong><\/p>\n<p>[\/et_pb_blurb][\/et_pb_column][\/et_pb_row][et_pb_row custom_padding_last_edited=\u00a0\u00bbon|phone\u00a0\u00bb _builder_version=\u00a0\u00bb4.16&Prime; background_color=\u00a0\u00bb#ffffff\u00a0\u00bb max_width=\u00a0\u00bb1200px\u00a0\u00bb custom_padding=\u00a0\u00bb29px|80px|28px|80px|false|false\u00a0\u00bb custom_padding_tablet=\u00a0\u00bb|80px||80px|false|true\u00a0\u00bb custom_padding_phone=\u00a0\u00bb40px|30px||30px||true\u00a0\u00bb box_shadow_style=\u00a0\u00bbpreset3&Prime; box_shadow_vertical=\u00a0\u00bb40px\u00a0\u00bb box_shadow_blur=\u00a0\u00bb100px\u00a0\u00bb box_shadow_spread=\u00a0\u00bb-30px\u00a0\u00bb box_shadow_color=\u00a0\u00bbrgba(0,0,0,0.22)\u00a0\u00bb use_custom_width=\u00a0\u00bbon\u00a0\u00bb custom_width_px=\u00a0\u00bb1200px\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][et_pb_column type=\u00a0\u00bb4_4&Prime; _builder_version=\u00a0\u00bb4.16&Prime; custom_padding=\u00a0\u00bb|||\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb custom_padding__hover=\u00a0\u00bb|||\u00a0\u00bb][et_pb_blurb title=\u00a0\u00bb912021 &#8211; 2023%93 &#8211; Projet de g\u00e9n\u00e9ration de mod\u00e8les Clcn4-rat (\u00a350,000)\u00a0\u00bb content_max_width=\u00a0\u00bb800px\u00a0\u00bb _builder_version=\u00a0\u00bb4.23.1&Prime; header_font=\u00a0\u00bb|700||on|||||\u00a0\u00bb header_font_size=\u00a0\u00bb17px\u00a0\u00bb header_line_height=\u00a0\u00bb2em\u00a0\u00bb body_font_size=\u00a0\u00bb17px\u00a0\u00bb body_line_height=\u00a0\u00bb2em\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb]<\/p>\n<p style=\"text-align: justify;\"><strong>Justification :<\/strong> Les efforts de recherche sur les troubles neurod\u00e9veloppementaux <em>li\u00e9s \u00e0 la CLCN4<\/em><em>(CLCN4-NDC<\/em>) ont \u00e9t\u00e9 entrav\u00e9s par l&rsquo;absence d&rsquo;un mod\u00e8le animal appropri\u00e9. Les lign\u00e9es de souris knock-out (KO) de <em>Clcn4<\/em> g\u00e9n\u00e9r\u00e9es pr\u00e9c\u00e9demment ne pr\u00e9sentaient pas de ph\u00e9notype manifeste (d\u00e9veloppement normal, pas de changements anatomiques dans le cerveau, ni d&rsquo;anomalies comportementales \u00e9videntes)<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC2878524\/\" target=\"_blank\" rel=\"noopener\">[1<\/a><a href=\"https:\/\/www.nature.com\/articles\/mp2016135\" target=\"_blank\" rel=\"noopener\">,2<\/a><a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC7196918\/\" target=\"_blank\" rel=\"noopener\">,3<\/a>]. Cela peut s&rsquo;expliquer par le fait que le g\u00e8ne <em>Clcn4<\/em> de la souris est autosomique et non li\u00e9 \u00e0 l&rsquo;X. Afin de surmonter cet obstacle, et \u00e9tant donn\u00e9 que le g\u00e8ne <em>Clcn4<\/em> se trouve sur le chromosome X chez le rat, ce qui signifie que le g\u00e8ne <em>Clcn4<\/em> lui-m\u00eame et son environnement dans le g\u00e9nome sont mieux conserv\u00e9s, la Cure CLCN4 a cherch\u00e9 \u00e0 g\u00e9n\u00e9rer un ou plusieurs mod\u00e8les de <em>CLCN4-NDC<\/em> chez le rat.   <\/p>\n<p style=\"text-align: justify;\"><strong>Objectif :<\/strong> L&rsquo;objectif de ce projet \u00e9tait de g\u00e9n\u00e9rer trois mod\u00e8les de <em>rats Clcn4<\/em>, \u00e0 savoir : i) un rat knock-out <em>Clcn4<\/em>; ii) un rat knock-out conditionnel <em>Clcn4<\/em>, et iii) une lign\u00e9e knock-in (KI) <em>Clcn4<\/em>, h\u00e9bergeant l&rsquo;\u00e9quivalent pour le rat de la variante p.(Ala555Val) <em>CLCN4<\/em>, la variante r\u00e9currente la plus courante avec un fort ph\u00e9notype de gain de fonction dans le mod\u00e8le de <em>Xenopus<\/em>.<\/p>\n<p style=\"text-align: justify;\"><strong>R\u00e9sultats :<\/strong> Gr\u00e2ce au financement fourni par Cure CLCN4 et ex\u00e9cut\u00e9 par l&rsquo;IGBMC (Institut de g\u00e9n\u00e9tique et de biologie mol\u00e9culaire et cellulaire, France), ce projet a \u00e9t\u00e9 men\u00e9 \u00e0 bien et les trois mod\u00e8les de rats sont disponibles sur le d\u00e9p\u00f4t INFRAFRONTIER. Pour plus de d\u00e9tails et pour savoir comment commander, veuillez nous envoyer un courriel \u00e0 l&rsquo;adresse suivante : info@cureclcn4.org <\/p>\n<p>[\/et_pb_blurb][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=\u00a0\u00bb1&Prime; admin_label=\u00a0\u00bbVideo\u00a0\u00bb _builder_version=\u00a0\u00bb4.16&Prime; background_color=\u00a0\u00bb#3a897e\u00a0\u00bb custom_padding=\u00a0\u00bb0|1px|0|0px|false|false\u00a0\u00bb collapsed=\u00a0\u00bboff\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][et_pb_row _builder_version=\u00a0\u00bb4.16&Prime; _module_preset=\u00a0\u00bbdefault\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][et_pb_column type=\u00a0\u00bb4_4&Prime; _builder_version=\u00a0\u00bb4.16&Prime; _module_preset=\u00a0\u00bbdefault\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][et_pb_divider show_divider=\u00a0\u00bboff\u00a0\u00bb _builder_version=\u00a0\u00bb4.16&Prime; _module_preset=\u00a0\u00bbdefault\u00a0\u00bb global_colors_info=\u00a0\u00bb{}\u00a0\u00bb][\/et_pb_divider][\/et_pb_column][\/et_pb_row][\/et_pb_section][et_pb_section fb_built=\u00a0\u00bb1&Prime; 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