{"id":43786,"date":"2026-10-03T03:30:49","date_gmt":"2026-10-03T03:30:49","guid":{"rendered":"https:\/\/harissa.com\/news-wp\/?p=43786"},"modified":"2026-10-03T03:30:49","modified_gmt":"2026-10-03T03:30:49","slug":"forme-genetique-de-la-maladie-de-creutzfeld-jacob","status":"publish","type":"post","link":"https:\/\/harissa.com\/news-wp\/forme-genetique-de-la-maladie-de-creutzfeld-jacob\/","title":{"rendered":"Forme genetique de la Maladie De Creutzfeld-Jacob"},"content":{"rendered":"<div>\n<div>\n<div>\n<div>\n<div>\n<p><b>Forme g\u00e9n\u00e9tique de la maladie de Creutzfeldt-jakob\u00a0<br \/> Lign\u00e9e juive \u00e0 haut risque<\/b><\/p>\n<\/div><\/div>\n<\/p><\/div>\n<\/p><\/div>\n<\/div>\n<p><b>Forme g\u00e9n\u00e9tique de la maladie de Creutzfeldt-jakob\u00a0<br \/> Lign\u00e9e juive \u00e0 haut risque\u00a0<\/p>\n<p> Les juifs d&rsquo;origine libyenne sont cent fois plus expos\u00e9s que la moyenne \u00e0 la forme g\u00e9n\u00e9tique de la maladie de Creutzfeldt-Jakob. Et les juifs tunisiens, cinq fois plus. Gros plan sur une maladie h\u00e9r\u00e9ditaire m\u00e9connue.\u00a0<\/p>\n<p> Fin mai, alerte en Guadeloupe. Une insulaire y serait la\u00a0 quatri\u00e8me victime de la forme humaine de la maladie de la vache folle. La patiente n&rsquo;a que 47 ans. Or, les formes classiques de la maladie de Creutzfeldt-Jakob (MCJ) touchent des sujets de plus de 55 ans. L&rsquo;\u00e2ge de la malade et les sympt\u00f4mes font redouter le nouveau variant (nv-MCJ).\u00a0<br \/> A moins qu&rsquo;il ne s&rsquo;agisse de la forme g\u00e9n\u00e9tique de la maladie, qui repr\u00e9sente 10 \u00e0 15 % des cas et menace tous les descendants d&rsquo;une m\u00eame famille. Comme le souligne Jean-Louis Laplanche, professeur au laboratoire de biochimie de l&rsquo;h\u00f4pital Lariboisi\u00e8re, \u00e0 Paris, \u00a0\u00bb pour l&rsquo;instant, nous n&rsquo;avons aucune id\u00e9e de la pr\u00e9valence de la mutation dans la population fran\u00e7aise \u00ab\u00a0. Autrement dit, personne ne sait combien d&rsquo;individus sont porteurs de ce g\u00e8ne mut\u00e9. Seule certitude : il existe des lign\u00e9es fragiles.\u00a0<\/p>\n<p> \u00a0\u00bb Chez les juifs d&rsquo;origine libyenne, l&rsquo;incidence de la maladie est 100 fois plus \u00e9lev\u00e9e que la moyenne, affirme le Dr Esther Kahana, neurologue \u00e0 l&rsquo;h\u00f4pital Barzilai d&rsquo;Ashkelon, en Isra\u00ebl. Chez ceux d&rsquo;origine tunisienne, elle est 4 \u00e0 5 fois plus importante. Les descendants des familles porteuses de la\u00a0 mutation ont une chance sur deux de d\u00e9velopper une MCJ. \u00a0\u00bb Des chiffres qui r\u00e9v\u00e8lent l&rsquo;importance du probl\u00e8me. En Isra\u00ebl, certains chercheurs ont m\u00eame demand\u00e9, au nom du principe de pr\u00e9caution, un test g\u00e9n\u00e9tique pour ces populations avant tout don d&rsquo;organe ou de sang. Sans suite.\u00a0<br \/> Outre le souci de limiter le risque sanitaire, le recensement des porteurs du g\u00e8ne mut\u00e9 serait pourtant d&rsquo;une grande utilit\u00e9. \u00a0\u00bb Ces cas h\u00e9r\u00e9ditaires peuvent surprendre car certains ont des sympt\u00f4mes neurologiques trompeurs, commente le Pr Laplanche. S&rsquo;ils sont tous li\u00e9s \u00e0 la mutation d&rsquo;un m\u00eame g\u00e8ne, celui codant la prot\u00e9ine prion, il existe vingt types de mutations diff\u00e9rentes et presque autant de tableaux cliniques. \u00a0\u00bb\u00a0<\/p>\n<p> Autre avantage d&rsquo;un d\u00e9nombrement : \u00a0\u00bb Il nous aiderait \u00e0 \u00e9laborer un certain nombre de tests, dont des \u00e9valuations psychologiques, afin de rep\u00e9rer les premiers signes neurologiques suspects et prendre en charge les malades le plus t\u00f4t possible \u00ab\u00a0, explique Nelly Zilber, \u00e9pid\u00e9miologiste \u00e0 l&rsquo;h\u00f4pital Hadassah de J\u00e9rusalem. Il apporterait aussi de riches informations sur l&rsquo;\u00e9tiologie des maladies \u00e0 prions. <\/p>\n<p> Car contrairement aux autres pathologies g\u00e9n\u00e9tiques, les MCJ familiales ne se d\u00e9clarent pas syst\u00e9matiquement en cas de mutation. Exemple : \u00a0\u00bb Nous ne savons pas pourquoi deux filles porteuses du g\u00e8ne mut\u00e9 meurent d&rsquo;une MCJ avant 60 ans, note le Pr Laplanche, alors que leur m\u00e8re porteuse de la m\u00eame anomalie va d\u00e9c\u00e9der \u00e0 90 ans sans aucun trouble neurologique. Un ou des facteurs inconnus retarderaient le d\u00e9veloppement de la maladie. \u00a0\u00bb L&rsquo;une des hypoth\u00e8ses avanc\u00e9es, celle d&rsquo;\u00e9l\u00e9ments environnementaux qui influent sur l&rsquo;expression de la mutation, fait l&rsquo;objet de recherches. Mettre en \u00e9vidence de tels param\u00e8tres serait une avanc\u00e9e consid\u00e9rable. \u00a0\u00bb Un point de d\u00e9part pour l&rsquo;\u00e9laboration d&rsquo;un traitement \u00ab\u00a0, esp\u00e8re Jean-Jacques Laplanche.\u00a0<\/p>\n<p> Linda Bendali\u00a0<\/p>\n<p> Sciences &amp; Avenir N\u00b0653\u00a0<br \/> &lt;<a href=\"mailto:sudnord@caramail.com\">sudnord@caramail.com<\/a>&gt;\u00a0<\/b><\/p>\n<hr\/>\n<blockquote>\n<blockquote><\/blockquote>\n<\/blockquote>\n<h6><\/h6>\n","protected":false},"excerpt":{"rendered":"<p>Forme g\u00e9n\u00e9tique de la maladie de Creutzfeldt-jakob\u00a0 Lign\u00e9e juive \u00e0 haut risque Forme g\u00e9n\u00e9tique de la maladie de Creutzfeldt-jakob\u00a0 Lign\u00e9e juive \u00e0 haut risque\u00a0 Les juifs d&rsquo;origine libyenne sont cent fois plus expos\u00e9s que la moyenne \u00e0 la forme g\u00e9n\u00e9tique de la maladie de Creutzfeldt-Jakob. Et les juifs tunisiens, cinq fois plus. Gros plan sur&hellip;&nbsp;<\/p>\n","protected":false},"author":0,"featured_media":23292,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"","neve_meta_content_width":0,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","footnotes":""},"categories":[16],"tags":[],"class_list":["post-43786","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-judaisme"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.6 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Forme genetique de la Maladie De Creutzfeld-Jacob | HARISSA.COM<\/title>\n<meta name=\"description\" content=\"Forme g\u00e9n\u00e9tique de la maladie de Creutzfeldt-jakob\u00a0 Lign\u00e9e juive \u00e0 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