{"id":1020,"date":"2017-08-24T02:33:56","date_gmt":"2017-08-24T02:33:56","guid":{"rendered":"http:\/\/stg-blogs.bmj.com\/jmg\/?p=1020"},"modified":"2017-08-24T02:33:56","modified_gmt":"2017-08-24T02:33:56","slug":"fabry-disease-characterisation-of-the-plasma-proteome-pre-and-post-enzyme-replacement-therapy","status":"publish","type":"post","link":"https:\/\/stg-blogs.bmj.com\/jmg\/2017\/08\/24\/fabry-disease-characterisation-of-the-plasma-proteome-pre-and-post-enzyme-replacement-therapy\/","title":{"rendered":"Fabry disease: characterisation of the plasma proteome pre- and post-enzyme replacement therapy"},"content":{"rendered":"<p>Fabry disease (FD) is characterized by the\u00a0progressive\u00a0accumulation of globotriaosylceramide (Gb3). Enzyme replacement therapy (ERT) clears this accumulation.\u00a0We analyzed plasma proteome profiles before and after\u00a0ERT\u00a0to characterize\u00a0its molecular pathology.\u00a0After\u00a0ERT,\u00a0the levels of proteins involved in inflammation, oxidative and ischemic injury, or complement activation were reduced significantly. In particular, we found out that inactivated complement C3b (iC3b) was significantly elevated in pre-ERT FD\u00a0plasma and it gradually decreased along ERT, comparable to the changes of Gb3.Our study indicates that C3-mediated complement activation might be altered in\u00a0FD\u00a0and\u00a0ERT\u00a0might promote its stabilization. (By Dr. Beom Hee Lee, <a href=\"http:\/\/jmg.bmj.com\/content\/early\/2017\/08\/23\/jmedgenet-2017-104704\">http:\/\/jmg.bmj.com\/content\/early\/2017\/08\/23\/jmedgenet-2017-104704<\/a> )<\/p>\n<p><a href=\"https:\/\/stg-blogs.bmj.com\/jmg\/files\/2017\/08\/8232.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone size-medium wp-image-1021\" src=\"https:\/\/stg-blogs.bmj.com\/jmg\/files\/2017\/08\/8232-222x300.jpg\" alt=\"\" width=\"222\" height=\"300\" srcset=\"https:\/\/stg-blogs.bmj.com\/jmg\/files\/2017\/08\/8232-222x300.jpg 222w, https:\/\/stg-blogs.bmj.com\/jmg\/files\/2017\/08\/8232-768x1039.jpg 768w, https:\/\/stg-blogs.bmj.com\/jmg\/files\/2017\/08\/8232-757x1024.jpg 757w, https:\/\/stg-blogs.bmj.com\/jmg\/files\/2017\/08\/8232-300x406.jpg 300w\" sizes=\"auto, (max-width: 222px) 100vw, 222px\" \/><\/a><!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Fabry disease (FD) is characterized by the\u00a0progressive\u00a0accumulation of globotriaosylceramide (Gb3). Enzyme replacement therapy (ERT) clears this accumulation.\u00a0We analyzed plasma proteome profiles before and after\u00a0ERT\u00a0to characterize\u00a0its molecular pathology.\u00a0After\u00a0ERT,\u00a0the levels of proteins involved in inflammation, oxidative and ischemic injury, or complement activation were reduced significantly. In particular, we found out that inactivated complement C3b (iC3b) was significantly [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/stg-blogs.bmj.com\/jmg\/2017\/08\/24\/fabry-disease-characterisation-of-the-plasma-proteome-pre-and-post-enzyme-replacement-therapy\/\">Read More&#8230;<\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_jetpack_memberships_contains_paid_content":false,"footnotes":""},"categories":[1],"tags":[],"class_list":["post-1020","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"jetpack_featured_media_url":"","jetpack_sharing_enabled":true,"_links":{"self":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/posts\/1020","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/comments?post=1020"}],"version-history":[{"count":0,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/posts\/1020\/revisions"}],"wp:attachment":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/media?parent=1020"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/categories?post=1020"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/tags?post=1020"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}