{"id":4141,"date":"2026-02-12T16:20:08","date_gmt":"2026-02-12T16:20:08","guid":{"rendered":"https:\/\/www.europesays.com\/be-fr\/4141\/"},"modified":"2026-02-12T16:20:08","modified_gmt":"2026-02-12T16:20:08","slug":"myopathie-et-maladie-de-pompe-maladies-lysosomales","status":"publish","type":"post","link":"https:\/\/www.europesays.com\/be-fr\/4141\/","title":{"rendered":"Myopathie et Maladie de Pompe | Maladies lysosomales"},"content":{"rendered":"<p class=\"MuiTypography-root MuiTypography-body1 css-u6l2z2-MuiTypography-root\">Les\u00a0maladies\u00a0lysosomales, ou maladies\u00a0de surcharge\u00a0lysosomale,\u202fregroupent\u00a0une soixantaine d\u2019affections\u00a0g\u00e9n\u00e9tiques, chez\u00a0l\u2019enfant\u00a0comme chez\u00a0l\u2019adulte.\u00a0Elles\u00a0ont en commun une\u00a0dysfonction\u00a0des\u00a0lysosomes. Comme des usines de transformation3, ces\u00a0minuscules\u00a0\u00e9l\u00e9ments\u00a0se\u00a0chargent\u00a0de d\u00e9grader\u00a0et de\u00a0recycler\u00a0les\u00a0\u00ab\u202fd\u00e9chets\u202f\u00bb\u00a0produits par l\u2019organisme. Pour ce faire, ils utilisent des enzymes sp\u00e9cifiques. Lorsque l\u2019une d\u2019elles manque ou fonctionne mal, les d\u00e9chets ne sont plus \u00e9limin\u00e9s. Ils s\u2019accumulent dans les cellules, perturbant l\u2019activit\u00e9 de divers organes, comme le c\u0153ur, les poumons, le foie, le cerveau, ou les muscles, en fonction des pathologies. C\u2019est pr\u00e9cis\u00e9ment cette surcharge qui donne son nom \u00e0 ces maladies.<\/p>\n<p class=\"MuiTypography-root MuiTypography-body1 css-nr8ku2-MuiTypography-root\">Chaque maladie lysosomale implique un g\u00e8ne diff\u00e9rent et concerne une enzyme particuli\u00e8re4. Lorsque les muscles sont touch\u00e9s, on parle de myopathie, caract\u00e9ris\u00e9e par une faiblesse musculaire progressive. Les muscles respiratoires et cardiaques peuvent \u00eatre atteints, ce qui explique la gravit\u00e9 potentielle de ces maladies.<\/p>\n","protected":false},"excerpt":{"rendered":"Les\u00a0maladies\u00a0lysosomales, ou maladies\u00a0de surcharge\u00a0lysosomale,\u202fregroupent\u00a0une soixantaine d\u2019affections\u00a0g\u00e9n\u00e9tiques, chez\u00a0l\u2019enfant\u00a0comme chez\u00a0l\u2019adulte.\u00a0Elles\u00a0ont en commun une\u00a0dysfonction\u00a0des\u00a0lysosomes. Comme des usines de transformation3, ces\u00a0minuscules\u00a0\u00e9l\u00e9ments\u00a0se\u00a0chargent\u00a0de d\u00e9grader\u00a0et&hellip;\n","protected":false},"author":2,"featured_media":4142,"comment_status":"","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[82],"tags":[12,13,18,17,3369,3365,3370,3362,2191,3360,86,3366,3359,3368,3367,3361,3358,3363,3364,87],"class_list":{"0":"post-4141","1":"post","2":"type-post","3":"status-publish","4":"format-standard","5":"has-post-thumbnail","7":"category-sante","8":"tag-be","9":"tag-be-fr","10":"tag-belgique","11":"tag-belgium","12":"tag-deficit-en-alpha-glucosidase","13":"tag-enzyme","14":"tag-enzymotherapie-substitutive","15":"tag-faiblesse-musculaire-progressive","16":"tag-glycogene","17":"tag-glycogenose-musculaire","18":"tag-health","19":"tag-lysosomale","20":"tag-maladie-de-pompe","21":"tag-maladie-genetique","22":"tag-maladie-rare","23":"tag-muscle","24":"tag-myopathie","25":"tag-myopathie-metabolique","26":"tag-respiratoire","27":"tag-sante"},"share_on_mastodon":{"url":"","error":""},"_links":{"self":[{"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/posts\/4141","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/comments?post=4141"}],"version-history":[{"count":0,"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/posts\/4141\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/media\/4142"}],"wp:attachment":[{"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/media?parent=4141"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/categories?post=4141"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.europesays.com\/be-fr\/wp-json\/wp\/v2\/tags?post=4141"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}