{"id":13392,"date":"2022-12-27T11:32:10","date_gmt":"2022-12-27T10:32:10","guid":{"rendered":"https:\/\/www.cutislaxa.org\/?p=13392"},"modified":"2023-01-09T11:34:11","modified_gmt":"2023-01-09T10:34:11","slug":"prof-dr-uwe-kornak-md-gottingen-allemagne-la-voie-secretoire-glycosylation-et-cl","status":"publish","type":"post","link":"https:\/\/www.cutislaxa.org\/fr\/prof-dr-uwe-kornak-md-gottingen-allemagne-la-voie-secretoire-glycosylation-et-cl\/","title":{"rendered":"Prof. Dr. Uwe Kornak, MD (G\u00f6ttingen, Allemagne): La Voie S\u00e9cr\u00e9toire, Glycosylation et CL"},"content":{"rendered":"<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone size-medium wp-image-13377 aligncenter\" src=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526-300x206.jpg\" alt=\"\" width=\"300\" height=\"206\" srcset=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526-300x206.jpg 300w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526-1030x706.jpg 1030w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526-768x526.jpg 768w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526-1536x1052.jpg 1536w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526-1500x1028.jpg 1500w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526-705x483.jpg 705w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_122526.jpg 2004w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/p>\n<p>Les prot\u00e9ines secr\u00e9t\u00e9es et les prot\u00e9ines \u00e0 membrane doivent passer par la voie s\u00e9cr\u00e9toire qui est non seulement responsable du transport vers le bon compartiment cellulaire mais aussi de la maturation et de la modification de ces prot\u00e9ines. La partie centrale de cette voie s\u00e9cr\u00e9toire est le compartiment Golgi qui consiste en plusieurs sacs de \u00a0membrane ressemblant \u00e0 des cr\u00eapes (cisternae).<\/p>\n<p style=\"text-align: center;\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-13380\" src=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-300x193.jpg\" alt=\"\" width=\"280\" height=\"180\" srcset=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-300x193.jpg 300w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-1030x662.jpg 1030w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-768x493.jpg 768w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-1536x987.jpg 1536w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-2048x1316.jpg 2048w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-1500x964.jpg 1500w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_123245-705x453.jpg 705w\" sizes=\"auto, (max-width: 280px) 100vw, 280px\" \/> <img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-13383\" src=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_124515-2-300x204.jpg\" alt=\"\" width=\"265\" height=\"180\" srcset=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_124515-2-300x204.jpg 300w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_124515-2-1030x701.jpg 1030w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_124515-2-768x522.jpg 768w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_124515-2-1500x1020.jpg 1500w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_124515-2-705x480.jpg 705w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_124515-2.jpg 1507w\" sizes=\"auto, (max-width: 265px) 100vw, 265px\" \/><\/p>\n<p>Une modification importante est la glycosylation qu\u2019on a trouv\u00e9 modifi\u00e9e dans tout un groupe de maladies cutis laxa, que l\u2019on appelle aussi maladies cong\u00e9nitales de la glycosylation (CDG).<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignnone size-medium wp-image-13386 aligncenter\" src=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_125310-300x190.jpg\" alt=\"\" width=\"300\" height=\"190\" srcset=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_125310-300x190.jpg 300w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_125310-1030x653.jpg 1030w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_125310-768x487.jpg 768w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_125310-705x447.jpg 705w, https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/IMG_20220915_125310.jpg 1397w\" sizes=\"auto, (max-width: 300px) 100vw, 300px\" \/><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Les prot\u00e9ines secr\u00e9t\u00e9es et les prot\u00e9ines \u00e0 membrane doivent passer par la voie s\u00e9cr\u00e9toire qui est non seulement responsable du transport vers le bon compartiment cellulaire mais aussi de la maturation et de la modification de ces prot\u00e9ines. La partie centrale de cette voie s\u00e9cr\u00e9toire est le compartiment Golgi qui consiste en plusieurs sacs de [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":13374,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[59,67,61,60,66,64],"tags":[],"class_list":["post-13392","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-number-mutations-fr","category-photos-fr","category-quoi-de-neuf","category-recherche","category-recherche-medecine-genetique","category-rencontres-evenements-et-manifestations"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v22.0 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Prof. Dr. Uwe Kornak, MD (G\u00f6ttingen, Allemagne): La Voie S\u00e9cr\u00e9toire, Glycosylation et CL - CUTIS LAXA INTERNATIONALE<\/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:\/\/www.cutislaxa.org\/202209-prof-dr-uwe-kornak_the-secretory-pathway-glycosylation-and-cutis-laxa\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Prof. Dr. Uwe Kornak, MD (G\u00f6ttingen, Allemagne): La Voie S\u00e9cr\u00e9toire, Glycosylation et CL - CUTIS LAXA INTERNATIONALE\" \/>\n<meta property=\"og:description\" content=\"Les prot\u00e9ines secr\u00e9t\u00e9es et les prot\u00e9ines \u00e0 membrane doivent passer par la voie s\u00e9cr\u00e9toire qui est non seulement responsable du transport vers le bon compartiment cellulaire mais aussi de la maturation et de la modification de ces prot\u00e9ines. La partie centrale de cette voie s\u00e9cr\u00e9toire est le compartiment Golgi qui consiste en plusieurs sacs de [&hellip;]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.cutislaxa.org\/202209-prof-dr-uwe-kornak_the-secretory-pathway-glycosylation-and-cutis-laxa\/\" \/>\n<meta property=\"og:site_name\" content=\"CUTIS LAXA INTERNATIONALE\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/CutisLaxaAssociation\/\" \/>\n<meta property=\"article:published_time\" content=\"2022-12-27T10:32:10+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2023-01-09T10:34:11+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.cutislaxa.org\/wp-content\/uploads\/2023\/01\/UWE.jpg\" \/>\n\t<meta property=\"og:image:width\" content=\"156\" \/>\n\t<meta property=\"og:image:height\" content=\"174\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/jpeg\" \/>\n<meta name=\"author\" content=\"Marie-Claude\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"\u00c9crit par\" \/>\n\t<meta name=\"twitter:data1\" content=\"Marie-Claude\" \/>\n\t<meta name=\"twitter:label2\" content=\"Dur\u00e9e de lecture estim\u00e9e\" \/>\n\t<meta name=\"twitter:data2\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\/\/www.cutislaxa.org\/202209-prof-dr-uwe-kornak_the-secretory-pathway-glycosylation-and-cutis-laxa\/#article\",\"isPartOf\":{\"@id\":\"https:\/\/www.cutislaxa.org\/202209-prof-dr-uwe-kornak_the-secretory-pathway-glycosylation-and-cutis-laxa\/\"},\"author\":{\"name\":\"Marie-Claude\",\"@id\":\"https:\/\/www.cutislaxa.org\/#\/schema\/person\/2b5e58dca5a6f412607f985f2823d045\"},\"headline\":\"Prof. Dr. Uwe Kornak, MD (G\u00f6ttingen, Allemagne): La Voie S\u00e9cr\u00e9toire, Glycosylation et CL\",\"datePublished\":\"2022-12-27T10:32:10+00:00\",\"dateModified\":\"2023-01-09T10:34:11+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\/\/www.cutislaxa.org\/202209-prof-dr-uwe-kornak_the-secretory-pathway-glycosylation-and-cutis-laxa\/\"},\"wordCount\":117,\"publisher\":{\"@id\":\"https:\/\/www.cutislaxa.org\/#organization\"},\"articleSection\":[\"Mutations\",\"Photos\",\"Quoi de neuf\",\"Recherche\",\"Recherche - 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