{"id":3216,"date":"2020-02-05T12:33:00","date_gmt":"2020-02-05T18:33:00","guid":{"rendered":"https:\/\/restruct-lgmd-2021.pantheonsite.io\/?p=3216"},"modified":"2021-02-19T18:30:53","modified_gmt":"2021-02-19T18:30:53","slug":"lgmd-researcher-dufour","status":"publish","type":"post","link":"https:\/\/www.lgmd-info.org\/zh\/researcher-interview\/2020\/02\/05\/lgmd-researcher-dufour\/","title":{"rendered":"LGMD \u7814\u7a76\u5458\uff1aAntoine Dufour"},"content":{"rendered":"<div class=\"fusion-fullwidth fullwidth-box fusion-builder-row-1 fusion-flex-container nonhundred-percent-fullwidth non-hundred-percent-height-scrolling\" style=\"--awb-border-radius-top-left:0px;--awb-border-radius-top-right:0px;--awb-border-radius-bottom-right:0px;--awb-border-radius-bottom-left:0px;--awb-flex-wrap:wrap;\" ><div class=\"fusion-builder-row fusion-row fusion-flex-align-items-flex-start fusion-flex-content-wrap\" style=\"max-width:calc( 1400px + 0px );margin-left: calc(-0px \/ 2 );margin-right: calc(-0px \/ 2 );\"><div class=\"fusion-layout-column fusion_builder_column fusion-builder-column-0 fusion_builder_column_1_1 1_1 fusion-flex-column\" style=\"--awb-bg-size:cover;--awb-width-large:100%;--awb-margin-top-large:0px;--awb-spacing-right-large:0px;--awb-margin-bottom-large:0px;--awb-spacing-left-large:0px;--awb-width-medium:100%;--awb-spacing-right-medium:0px;--awb-spacing-left-medium:0px;--awb-width-small:100%;--awb-spacing-right-small:0px;--awb-spacing-left-small:0px;\"><div class=\"fusion-column-wrapper fusion-flex-justify-content-flex-start fusion-content-layout-column\"><div class=\"fusion-text fusion-text-1\"><figure class=\"wp-block-image size-large\"><img decoding=\"async\" class=\"lazyload wp-image-3217\" src=\"data:image\/svg+xml,%3Csvg%20xmlns%3D%27http%3A%2F%2Fwww.w3.org%2F2000%2Fsvg%27%20width%3D%27960%27%20height%3D%27720%27%20viewBox%3D%270%200%20960%20720%27%3E%3Crect%20width%3D%27960%27%20height%3D%27720%27%20fill-opacity%3D%220%22%2F%3E%3C%2Fsvg%3E\" data-orig-src=\"https:\/\/www.lgmd-info.org\/wp-content\/uploads\/2020\/02\/Dufour-Antoine-1.png\" alt=\"\" \/><\/figure>\n<p>&nbsp;<\/p>\n<h2 class=\"wp-block-heading\">\"LGMD \u805a\u7126\u7814\u7a76\"<\/h2>\n<p>&nbsp;<\/p>\n<p>\u7814\u7a76\u5458   \u5b89\u6258\u4e07-\u675c\u5bcc\u5c14<\/p>\n<p>&nbsp;<\/p>\n<p>\u5de5\u4f5c\u5355\u4f4d\uff1a \u5361\u5c14\u52a0\u91cc\u5927\u5b66  \u5361\u5c14\u52a0\u91cc\u5927\u5b66<\/p>\n<p>&nbsp;<\/p>\n<p>\u89d2\u8272\u6216\u804c\u4f4d\uff1a  \u52a9\u7406\u6559\u6388<\/p>\n<p>&nbsp;<\/p>\n<p><strong>W<\/strong><strong>\u60a8\u63a5\u53d7\u8fc7\u54ea\u4e9b\u6559\u80b2\u548c\u57f9\u8bad\u624d\u80fd\u62c5\u4efb\u76ee\u524d\u7684\u804c\u4f4d\uff1f<\/strong><\/p>\n<p>&nbsp;<\/p>\n<p>\u7ebd\u7ea6\u5dde\u7acb\u5927\u5b66\u5965\u65af\u5a01\u6208\u5206\u6821\u6587\u5b66\u58eb<\/p>\n<p>&nbsp;<\/p>\n<p>\u7ebd\u7ea6\u77f3\u6eaa\u5927\u5b66\u7406\u5b66\u7855\u58eb\u548c\u535a\u58eb\u5b66\u4f4d<\/p>\n<p>&nbsp;<\/p>\n<p>\u52a0\u62ff\u5927\u4e0d\u5217\u98a0\u54e5\u4f26\u6bd4\u4e9a\u5927\u5b66\u535a\u58eb\u540e\u5956\u5b66\u91d1<\/p>\n<p>&nbsp;<\/p>\n<p><strong>\u662f\u4ec0\u4e48\u4fc3\u4f7f\u60a8\u4ece\u4e8b\u7814\u7a76\u5de5\u4f5c\uff0c\u5c24\u5176\u662f\u7814\u7a76\u808c\u8089\u840e\u7f29\u75c7\uff1f<\/strong><\/p>\n<p>&nbsp;<\/p>\n<p>2 \u4e2a\u539f\u56e0<\/p>\n<p>&nbsp;<\/p>\n<p>1- \u6211\u7814\u7a76\u7684\u5206\u5b50\u526a\u5200\u88ab\u79f0\u4e3a\u86cb\u767d\u9176\uff0c\u5b83\u4eec\u5728\u751f\u6d3b\u3001\u751f\u7269\u529f\u80fd\u548c\u5404\u79cd\u808c\u8089\u840e\u7f29\u75c7\u4e2d\u5177\u6709\u591a\u79cd\u529f\u80fd\u3002\u80a2\u8170\u808c\u8425\u517b\u4e0d\u826f\u75c7 2A \u578b\uff08LGMD2A\uff09\u662f\u7531\u4e00\u79cd\u79f0\u4e3a Calpain-3 \u7684\u86cb\u767d\u9176\u7a81\u53d8\u5f15\u8d77\u7684\u3002\u6211\u6b63\u81f4\u529b\u4e8e\u66f4\u597d\u5730\u4e86\u89e3\u5b83\u5728\u808c\u8089\u751f\u7269\u5b66\u4e2d\u7684\u529f\u80fd\u3002<\/p>\n<p>&nbsp;<\/p>\n<p>2- \u6211\u7684\u5927\u8868\u54e5\u60a3\u6709\u675c\u5174\u6c0f\u808c\u8089\u840e\u7f29\u75c7\uff0c\u6211\u4ece\u5c0f\u5c31\u5e2e\u4ed6\u505a\u5404\u79cd\u7b80\u5355\u7684\u5de5\u4f5c\uff0c\u968f\u7740\u75c5\u60c5\u7684\u6076\u5316\uff0c\u4ed6\u5df2\u7ecf\u4e0d\u80fd\u518d\u505a\u8fd9\u4e9b\u5de5\u4f5c\u4e86\u3002\u6211\u5f53\u65f6\u8fd8\u662f\u4e2a\u5b69\u5b50\/\u5c11\u5e74\uff08\u4ed6\u5728\u6211 14 \u5c81\u65f6\u53bb\u4e16\uff09\uff0c\u4f46\u8fd9\u7279\u522b\u51b3\u5b9a\u4e86\u6211\u7684\u804c\u4e1a\u9009\u62e9\u3002\u6211\u4e00\u76f4\u60f3\u6210\u4e3a\u4e00\u540d\u79d1\u5b66\u5bb6\uff0c\u7814\u7a76\u591a\u53d1\u6027\u786c\u5316\u75c7\u60a3\u8005\u808c\u8089\u529f\u80fd\u7f3a\u9677\u7684\u7279\u5f81\uff0c\u66f4\u597d\u5730\u4e86\u89e3\u8fd9\u79cd\u75be\u75c5\u662f\u5982\u4f55\u53d1\u751f\u7684\u3002\u7ecf\u8fc7\u6570\u5341\u5e74\u7684\u7814\u7a76\uff0c\u5173\u4e8e\u591a\u53d1\u6027\u786c\u5316\u75c7\u4ecd\u6709\u8bb8\u591a\u8c1c\u56e2\uff0c\u6211\u89c9\u5f97\u8fd9\u5f88\u5438\u5f15\u4eba\u3002<\/p>\n<p>&nbsp;<\/p>\n<p><strong>\u60a8\u6b63\u5728\u7814\u7a76\u54ea\u4e9b\u8bfe\u9898\uff1f<\/strong><\/p>\n<p>&nbsp;<\/p>\n<p>\u6211\u7814\u7a76\u5404\u79cd\u75be\u75c5\uff08\u5305\u62ec\u808c\u8089\u840e\u7f29\u75c7\u3001\u5173\u8282\u708e\u3001\u80c3\u80a0\u9053\u75be\u75c5\u548c\u764c\u75c7\uff09\u4e2d\u88ab\u79f0\u4e3a\u86cb\u767d\u9176\u7684\u5206\u5b50\u526a\u5200\u3002\u6211\u4eec\u91c7\u7528\u7684\u65b9\u6cd5\u53ef\u4ee5\u68c0\u6d4b\u548c\u8ddf\u8e2a\u6570\u5343\u79cd\u86cb\u767d\u8d28\u3002\u8fd9\u4f7f\u6211\u4eec\u80fd\u591f\u5728\u7cfb\u7edf\u5c42\u9762\u4e0a\u89c2\u5bdf\u75be\u75c5\u72b6\u51b5\uff0c\u5e76\u540c\u65f6\u76d1\u6d4b\u591a\u79cd\u53d8\u5316\u3002\u6211\u4eec\u5e94\u7528\u6211\u4eec\u7684\u6280\u672f\u6765\u63cf\u8ff0\u9499\u86cb\u767d\u9176-3\u7684\u7279\u6027\uff0c\u5f53\u8be5\u86cb\u767d\u9176-3\u7f3a\u9677\u65f6\uff0c\u4f1a\u76f4\u63a5\u5bfc\u81f4LGMD2A\u3002<\/p>\n<p>&nbsp;<\/p>\n<p><strong>\u60a8\u7684\u5de5\u4f5c\u5c06\u5982\u4f55\u5e2e\u52a9\u75c5\u4eba\uff1f\u5b83\u7684\u79d1\u5b66\u6027\u66f4\u5f3a\uff0c\u8fd8\u662f\u53ef\u80fd\u6210\u4e3a LGMD \u6216\u4e00\u822c\u533b\u5b66\u535a\u58eb\u7684\u6cbb\u7597\u65b9\u6cd5\uff1f<\/strong><\/p>\n<p>&nbsp;<\/p>\n<p>\u6211\u4eec\u7684\u5de5\u4f5c\u5177\u6709\u79d1\u5b66\u6027\uff0c\u5c06\u6709\u52a9\u4e8e\u63cf\u8ff0\u6240\u6709\u53d1\u751f\u53d8\u5316\u3001\u53d7\u5230\u5f71\u54cd\u548c\u5bfc\u81f4 LGMDs \u7684\u86cb\u767d\u8d28\u7684\u7279\u5f81\u3002\u5c3d\u7ba1\u8fdb\u884c\u4e86\u6570\u5341\u5e74\u7684\u7814\u7a76\uff0c\u4f46\u6211\u4eec\u5bf9\u591a\u53d1\u6027\u786c\u5316\u75c7\u7684\u4e86\u89e3\u8fd8\u5f88\u72ed\u7a84\uff0c\u800c\u6211\u4eec\u7684\u5de5\u4f5c\u63d0\u4f9b\u4e86\u65e0\u504f\u89c1\u7684\u65b9\u6cd5\u6765\u8ddf\u8e2a\u6570\u5343\u79cd\u53d8\u5316\uff0c\u5e76\u786e\u5b9a\u5728\u591a\u53d1\u6027\u786c\u5316\u75c7\u4e2d\u4ecd\u672a\u786e\u5b9a\u7684\u65b0\u578b\u836f\u7269\u9776\u70b9\u3002<\/p>\n<p>&nbsp;<\/p>\n<p><strong>\u60a8\u5e0c\u671b\u60a3\u8005\u548c\u5176\u4ed6\u5bf9 LGMD 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Dufour - LGMD Awareness Foundation<\/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.lgmd-info.org\/zh\/researcher-interview\/2020\/02\/05\/lgmd-researcher-dufour\/\" \/>\n<meta property=\"og:locale\" content=\"zh_CN\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"LGMD RESEARCHER: Antoine Dufour - LGMD Awareness Foundation\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.lgmd-info.org\/zh\/researcher-interview\/2020\/02\/05\/lgmd-researcher-dufour\/\" \/>\n<meta property=\"og:site_name\" content=\"LGMD Awareness Foundation\" \/>\n<meta property=\"article:publisher\" content=\"https:\/\/www.facebook.com\/LGMDawareness\/\" \/>\n<meta property=\"article:published_time\" content=\"2020-02-05T18:33:00+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2021-02-19T18:30:53+00:00\" \/>\n<meta property=\"og:image\" content=\"https:\/\/www.lgmd-info.org\/wp-content\/uploads\/2020\/02\/Dufour-Antoine-1.png\" \/>\n\t<meta property=\"og:image:width\" content=\"960\" \/>\n\t<meta property=\"og:image:height\" content=\"720\" \/>\n\t<meta property=\"og:image:type\" content=\"image\/png\" \/>\n<meta name=\"author\" content=\"dooley\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@LgmdAwareness\" \/>\n<meta name=\"twitter:site\" content=\"@LgmdAwareness\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"dooley\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"3 minutes\" \/>\n<script type=\"application\/ld+json\" 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