{"id":17216,"date":"2020-10-22T00:00:00","date_gmt":"2020-10-22T00:00:00","guid":{"rendered":"https:\/\/hdbuzzwp.codev.codev\/?p=17216"},"modified":"2020-12-30T00:00:00","modified_gmt":"2020-12-30T00:00:00","slug":"294-2","status":"publish","type":"post","link":"https:\/\/ko.hdbuzz.net\/294-2\/","title":{"rendered":"\ub2e4\ub978 \uc2e0\uacbd\uc9c8\ud658\uc5d0 \ub300\ud55c \uce58\ub8cc\uc57d\ubb3c\uc774 \ud5cc\ud305\ud134 \ubcd1 \ud658\uc790\uc5d0\uac8c\ub3c4 \ud6a8\uacfc\uac00 \uc788\uc744 \uc218 \uc788\ub2e4"},"content":{"rendered":"\nSMA \ud658\uc790\ub4e4\uc744 \uc704\ud55c branaplam\uc774\ub77c\ub294 \uc57d\uc744 \uac1c\ubc1c\ud558\ub358 \uc911, \uc81c\uc57d\ud68c\uc0ac Novartis\ub294 \ud5cc\ud305\ud134\ubcd1 \ud658\uc790\ub4e4\uc744 \uc704\ud55c \uc57d\uc774 \ub420 \uc218\ub3c4 \uc788\ub2e4\ub294 \uac83\uc744 \ubc1c\uacac\ud588\uc2b5\ub2c8\ub2e4. FDA\ub294 \ud76c\uadc0\uc758\uc57d\ud488 \uc9c0\uc815\uc774\ub77c\ub294 \ud2b9\ubcc4\ud55c \uc9c0\uc704\ub97c \ubd80\uc5ec\ud588\uc2b5\ub2c8\ub2e4.<\/p>\n\n\n\n\n
Huntington \uc218\uce58\ub97c \ub0ae\uc8fc\uae30 \uc704\ud55c \ud604\uc874\ud558\ub294 \uc57d?<\/h2>\n\n\n\n\n
\uc81c\uc57d\ud68c\uc0ac Novartis\ub294 \ubbf8\uad6d \uc2dd\ud488\uc758\uc57d\uad6d(FDA)\uc774 \ud5cc\ud305\ud134\ubcd1 \uce58\ub8cc\ub97c \uc704\ud574 \ud76c\uadc0\uc758\uc57d\ud488 \uc9c0\uc704\ub97c \ubd80\uc5ec\ud588\ub2e4\uace0 \ubc1c\ud45c\ud588\uc2b5\ub2c8\ub2e4.<\/p>\n\n\n\n\n
Branaplam\uc740 \uc6d0\ub798 \uc2e0\uacbd \uc9c8\ud658\uc758 \uc77c\uc885\uc778 \ucc99\ucd94 \uadfc\uc721 \uc704\ucd95(SMA)\uc758 \uce58\ub8cc\ub97c \uc704\ud574 \uac1c\ubc1c\ub418\uc5c8\uc73c\uba70 \uc774\ub7ec\ud55c \ubaa9\uc801\uc73c\ub85c \uc784\uc0c1 \uc870\uc0ac\ub97c \ubc1b\uace0 \uc788\uc2b5\ub2c8\ub2e4. Branaplam\ub294 \uc720\uc804\uc801 \uba54\uc2dc\uc9c0\uac00 \uc138\ud3ec\uc5d0\uc11c \ucc98\ub9ac\ub418\ub294 \ubc29\ubc95\uc744 \uac04\uc12d\ud568\uc73c\ub85c\uc368 \uc791\uc6a9\ud558\uace0 SMN2\ub77c\uace0 \ubd88\ub9ac\ub294 \ub2e8\ubc31\uc9c8\uc758 \uc218\uce58\ub97c \uc99d\uac00\uc2dc\ud0ac \uc218 \uc788\uc2b5\ub2c8\ub2e4. SMN2\uc758 \ubd80\uc2a4\ud305\uc740 \ub9ce\uc740 \uacbd\uc6b0\uc5d0 \uc9c8\ubcd1\uc758 \uadfc\ubcf8\uc801\uc778 \uc6d0\uc778\uc778 \ub0ae\uc740 \uc218\uce58\uc758 SMN2 \ub2e8\ubc31\uc9c8\uc744 \uac00\uc9c4 SMA \ud658\uc790\ub4e4\uc744 \ub3d5\uc2b5\ub2c8\ub2e4.<\/p>\n\n\n\n\n
\ud765\ubbf8\ub86d\uac8c\ub3c4, branaplam\uc740 \ub610\ud55c \ud5cc\ud305\ud134 \uc720\uc804\uc790 \uba54\uc2dc\uc9c0\uc640 \ud5cc\ud305\ud134 \ub2e8\ubc31\uc9c8\uc758 \uc218\uce58\ub97c \uac10\uc18c\uc2dc\ud0a4\ub294 \uac83\uc73c\ub85c \ubcf4\uc785\ub2c8\ub2e4. \uc774 \ubc29\ubc95\uc774 \ud5cc\ud305\ud134 \uce58\ub8cc\ubc95\ub4e4\uc774 \ubaa8\ub450 \ubaa9\ud45c\ub85c \ud558\ub294 \uac83\uc774\uace0 \ub9ce\uc740 \uc5f0\uad6c\uc790\ub4e4\uacfc \ud68c\uc0ac\ub4e4\uc774 \ud5cc\ud305\ud134\uc758 \ubcd1\uc744 \uce58\ub8cc\ud558\ub824\uace0 \ud558\ub294 \ubc29\ubc95\uc785\ub2c8\ub2e4.<\/p>\n\n\n\n\n
\ud604\uc7ac \uc784\uc0c1 \uc2dc\ud5d8\uc5d0 \uc788\ub294 \ud5cc\ud305\ud134 \uc218\uce58 \ud558\uac15 \uc694\ubc95\uc740 \ucc99\ucd94 \uc8fc\uc0ac\ub098 \ub1cc \uc218\uc220\uc744 \ud1b5\ud574 \uc8fc\uc5b4\uc838\uc57c \ud569\ub2c8\ub2e4. \uc774\uac83\ub4e4\uacfc \ub2ec\ub9ac, branaplam\uc740 \uc791\uc740 \ubd84\uc790\uc758 \uc57d\uc73c\ub85c, \uadf8\uac83\uc740 \uc54c\uc57d\uc73c\ub85c \ubcf5\uc6a9\ub420 \uc218 \uc788\ub2e4\ub294 \uac83\uc744 \uc758\ubbf8\ud569\ub2c8\ub2e4. \ub9cc\uc57d Novartis\uac00 branaplam\uc774 HD\ub97c \uc704\ud55c \ud6a8\uacfc\uc801\uc778 \uce58\ub8cc\ubc95\uc774\ub77c\ub294 \uac83\uc744 \ubcf4\uc5ec\uc904 \uc218 \uc788\ub2e4\uba74, \uc774\uac83\uc740 HD \ud658\uc790\ub4e4\uc744 \uc704\ud574 \ub354 \ud3b8\ub9ac\ud55c \uc54c\uc57d\uc73c\ub85c \uc8fc\uc5b4\uc9c8 \uc218 \uc788\ub2e4\ub294 \uac83\uc744 \uc758\ubbf8\ud560 \uac83\uc785\ub2c8\ub2e4!<\/p>\n\n\n\n\n
\ud76c\uadc0\uc758\uc57d\ud488 \uc9c0\uc815 – \uadf8 \ub2e4\uc74c\uc740 \ubb34\uc5c7\uc785\ub2c8\uae4c?<\/h2>\n\n\n\n\n
\ud76c\uadc0\uc758\uc57d\ud488 \uc9c0\uc704\ub97c \ubd80\uc5ec\ud558\ub824\ub294 FDA\uc758 \uacb0\uc815\uc740 \ubcf4\ud1b5 \ud5cc\ud305\ud134 \ubcd1\uacfc \uac19\uc740 \ud76c\uadc0 \uc9c8\ubcd1\uc758 \uce58\ub8cc\ub97c \uc704\ud55c \uc57d\uc774 \uac1c\ubc1c\ub418\uace0 \uc788\uc744 \ub54c \ub0b4\ub824\uc9d1\ub2c8\ub2e4. \ud76c\uadc0 \uc57d\ubb3c \uc0c1\ud0dc\ub97c \ud5c8\uac00\ud558\ub294 \uac83\uc740, \uc774 \uacbd\uc6b0 Novartis\uc5d0\uac8c, \uc18c\uc218\uc758 \ud658\uc790\ub9cc \uc788\ub294 \uc9c8\ubcd1\uc5d0 \ub300\ud55c \uc0c8\ub85c\uc6b4 \uc758\uc57d\ud488\uc758 \uac1c\ubc1c\uc744 \uc7a5\ub824\ud558\uae30 \uc704\ud574 \ud61c\ud0dd\uacfc \uc778\uc13c\ud2f0\ube0c\ub97c \uc81c\uacf5\ud560 \uc218 \uc788\uc2b5\ub2c8\ub2e4.<\/p>\n\n\n\n\n
HD \ud658\uc790\ub4e4\uc744 \uce58\ub8cc\ud558\uae30 \uc704\ud574 branaplam\uc744 \uc0ac\uc6a9\ud558\ub294 \uac83\uc5d0 \ub300\ud574 \uacfc\ud559\uc790\ub4e4\uc774 \uc774\ud574\ud558\uc9c0 \ubabb\ud558\ub294 \uac83\uc774 \uc5ec\uc804\ud788 \ub9ce\uc774 \uc788\uc2b5\ub2c8\ub2e4. \uc54c\uc57d \uae30\ubc18\uc758 \uce58\ub8cc\ub294 \ub1cc\ubfd0\ub9cc \uc544\ub2c8\ub77c \uc804\uc2e0\uc5d0 \ud6a8\uacfc\uac00 \uc788\uc744 \uac83\uc73c\ub85c \uc608\uc0c1\ub418\uace0 \ud5cc\ud305\ud134 \uc218\uce58\ub97c \uc904\uc774\ub294 \uac83\uc774 \uc5b4\ub514\uc5d0\uc11c\ub4e0 \uc5b4\ub5a4 \uc601\ud5a5\uc744 \ubbf8\uce60\uc9c0 \ub9e4\uc6b0 \uba85\ud655\ud558\uc9c0 \uc54a\uc2b5\ub2c8\ub2e4. \uac8c\ub2e4\uac00, \uc6b0\ub9ac\ub294 \uc774\ubbf8 branaplam\uc774 SMN2\uc640 \ud5cc\ud305\ud134 \ub2e8\ubc31\uc9c8\uc744 \ub458\ub2e4 \ubc14\uafc0 \uc218 \uc788\ub2e4\ub294 \uac83\uc744 \uc54c\uace0 \uc788\uc2b5\ub2c8\ub2e4. \uadf8\ub798\uc11c \uc544\ub9c8 \ub2e4\ub978 \ub2e8\ubc31\uc9c8\ub4e4\uc758 \uc218\uce58\ub3c4 branaplam \uce58\ub8cc\uc5d0 \uc758\ud574\uc11c\ub3c4 \ubc14\ub014\uc9c0\ub3c4 \ubaa8\ub985\ub2c8\ub2e4. \uacfc\ud559\uc790\ub4e4\uc774 \uc57d\ubb3c\uc758 “\ud589\ub3d9 \uba54\ucee4\ub2c8\uc998"\uc774\ub77c\uace0 \ubd80\ub974\ub294 \uac83\uc5d0 \ub300\ud55c \uc644\uc804\ud558\uace0 \uc815\ud655\ud55c \uc774\ud574\uac00 \uc5c6\ub2e4\uba74, \uc774\uac83\uc740 \uc5b4\ub5bb\uac8c branaplam \uce58\ub8cc\uac00 HD \uc99d\uc0c1\uc744 \ubc14\uafc0 \uc218 \uc788\ub294\uc9c0\ub97c \uc815\ud655\ud788 \uc54c\uc544\ub0b4\ub294 \uac83\uc744 \ubcf5\uc7a1\ud558\uac8c \ub9cc\ub4e4 \uc218 \uc788\uc2b5\ub2c8\ub2e4. \uc88b\uc740 \uc18c\uc2dd\uc740 \uadf8 \uc57d\uc774 SMA \ud658\uc790\ub4e4\uc758 \uc791\uc740 \uadf8\ub8f9\uc5d0\uc11c \uc774\ubbf8 \uc548\uc804\ud55c \uac83\uc73c\ub85c \ub4dc\ub7ec\ub0ac\ub2e4\ub294 \uac83\uc785\ub2c8\ub2e4.<\/p>\n\n\n\n\n
Novartis\ub294 \ud604\uc7ac 2021\ub144\uc5d0 \ucd9c\uc2dc \uc608\uc815\uc778 HD \ud658\uc790\ub97c \uc704\ud55c \uc784\uc0c1\uc2e4\ud5d8 2\uc0c1\uc744 \uc124\uacc4\ud558\uace0 \uc788\uc2b5\ub2c8\ub2e4. \uace7 \uc0c8\ub85c\uc6b4 \uc18c\uc2dd\uc744 \uc804\ud574 \ub4dc\ub9ac\uae38 \ubc14\ub78d\ub2c8\ub2e4!<\/p>\n\n\n","protected":false},"excerpt":{"rendered":"
\uc81c\uc57d \ud68c\uc0ac Novartis\ub294 SMA \ud658\uc790\ub97c \uc704\ud55c branaplam \uc57d\uc744 \uac1c\ubc1c\ud558\ub294 \ub3d9\uc548 HD \ud658\uc790\uc5d0\uac8c \ud6a8\uacfc\uac00 \uc788\uc744 \uc218 \uc788\uc74c\uc744 \ubc1c\uacac\ud588\uc2b5\ub2c8\ub2e4. FDA\ub294 \ud76c\uadc0 \uc758\uc57d\ud488 \uc9c0\uc815\uc774\ub77c\ub294 \ud2b9\ubcc4 \uc9c0\uc704\ub97c \ubd80\uc5ec\ud588\uc2b5\ub2c8\ub2e4.<\/p>\n","protected":false},"author":1,"featured_media":20491,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"_jetpack_memberships_contains_paid_content":false,"footnotes":""},"categories":[],"class_list":["post-17216","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry"],"jetpack_featured_media_url":"https:\/\/i0.wp.com\/ko.hdbuzz.net\/wp-content\/uploads\/2020\/10\/codev-2020-10-22-69dac3dfef-shutterstock_1317151139-scaled.jpg?fit=2560%2C1920&quality=89&ssl=1","acf":{"writers":[{"ID":4202,"user_firstname":"Rachel","user_lastname":"Harding","nickname":"Dr Rachel Harding","user_nicename":"rachelharding","display_name":"Dr Rachel Harding","user_email":"rachel.harding@utoronto.ca","user_url":"","user_registered":"2025-05-12 17:22:01","user_description":"Rachel Harding is an Assistant Professor at the Department of Pharmacology and Toxicology and a Principal Investigator at the Structural Genomics Consortium. Rachel completed her undergraduate and DPhil in structural biology at the University of Oxford, before moving to the University of Toronto for her postdoctoral training. A key focus of Rachel\u2019s research is the huntingtin protein, mutated in people with Huntington\u2019s disease. Huntington\u2019s is a devastating, incurable, genetic, neurodegenerative disease caused by a CAG-tract expansion in the Huntingtin gene. Rachel studies the structure-function of the huntingtin protein in both its wildtype and disease forms, with a view to better understand the mechanisms of disease, as well as trying to find new avenues of therapeutic intervention.","user_avatar":"
"}],"editors":[{"ID":4130,"user_firstname":"Leora","user_lastname":"Fox","nickname":"Dr Leora Fox","user_nicename":"leorafox","display_name":"Dr Leora Fox","user_email":"leora.fox@gmail.com","user_url":"","user_registered":"2025-05-12 17:21:54","user_description":"Leora Fox trained as a Huntington's Disease scientist, and obtained her PhD in 2016 at Columbia University, where she studied HD mouse models and how brain cells dispose of huntingtin protein. After gaining experience in Medical Communications she worked on research and patient engagement programs at the Huntington's Disease Society of America from 2017-2024. Dr. Fox continues to support the HD research community through her work at Fox Scientific Consulting. She is passionate about communicating science about HD and other brain diseases so that families understand recent progress, can make informed decisions about participating in clinical research, and are supported around research news.","user_avatar":"
"}],"translators":[4178],"sources":null,"conflicts":"\uc800\uc790\ub294 \uc5b4\ub5a0\ud55c \ubb38\uc81c\uac00 \ub420 \uc774\ud574\uad00\uacc4\ub97c \uac00\uc9c0\uace0 \uc788\uc9c0 \uc54a\uc2b5\ub2c8\ub2e4. "},"jetpack_sharing_enabled":true,"_links":{"self":[{"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/posts\/17216","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/comments?post=17216"}],"version-history":[{"count":0,"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/posts\/17216\/revisions"}],"acf:user":[{"embeddable":true,"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/users\/4178"},{"embeddable":true,"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/users\/Array"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/media\/20491"}],"wp:attachment":[{"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/media?parent=17216"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/ko.hdbuzz.net\/wp-json\/wp\/v2\/categories?post=17216"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}