{"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\/ru\/researcher-interview\/2020\/02\/05\/lgmd-researcher-dufour\/","title":{"rendered":"\u0418\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u0442\u0435\u043b\u044c LGMD: \u0410\u043d\u0442\u0443\u0430\u043d \u0414\u044e\u0444\u0443\u0440"},"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 \u0412 \u0426\u0415\u041d\u0422\u0420\u0415 \u0412\u041d\u0418\u041c\u0410\u041d\u0418\u042f - \u0418\u0421\u0421\u041b\u0415\u0414\u041e\u0412\u0410\u041d\u0418\u042f\"<\/h2>\n<p>&nbsp;<\/p>\n<p>\u0418\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u0442\u0435\u043b\u044c:   \u0410\u043d\u0442\u0443\u0430\u043d \u0414\u044e\u0444\u0443\u0440<\/p>\n<p>&nbsp;<\/p>\n<p>\u041c\u0435\u0441\u0442\u043e \u0440\u0430\u0431\u043e\u0442\u044b:  \u0423\u043d\u0438\u0432\u0435\u0440\u0441\u0438\u0442\u0435\u0442 \u041a\u0430\u043b\u0433\u0430\u0440\u0438<\/p>\n<p>&nbsp;<\/p>\n<p>\u0420\u043e\u043b\u044c \u0438\u043b\u0438 \u0434\u043e\u043b\u0436\u043d\u043e\u0441\u0442\u044c:  \u0414\u043e\u0446\u0435\u043d\u0442<\/p>\n<p>&nbsp;<\/p>\n<p><strong>W<\/strong><strong>\u043a\u0430\u043a\u043e\u0435 \u043e\u0431\u0440\u0430\u0437\u043e\u0432\u0430\u043d\u0438\u0435 \u0438 \u043f\u043e\u0434\u0433\u043e\u0442\u043e\u0432\u043a\u0443 \u0432\u044b \u043f\u0440\u043e\u0448\u043b\u0438, \u0447\u0442\u043e\u0431\u044b \u0437\u0430\u043d\u044f\u0442\u044c \u0441\u0432\u043e\u044e \u043d\u044b\u043d\u0435\u0448\u043d\u044e\u044e \u0434\u043e\u043b\u0436\u043d\u043e\u0441\u0442\u044c?<\/strong><\/p>\n<p>&nbsp;<\/p>\n<p>\u0411\u0430\u043a\u0430\u043b\u0430\u0432\u0440, SUNY Oswego, \u041d\u044c\u044e-\u0419\u043e\u0440\u043a<\/p>\n<p>&nbsp;<\/p>\n<p>\u041c\u0430\u0433\u0438\u0441\u0442\u0440 \u0438 \u0434\u043e\u043a\u0442\u043e\u0440 \u0444\u0438\u043b\u043e\u0441\u043e\u0444\u0438\u0438, \u0423\u043d\u0438\u0432\u0435\u0440\u0441\u0438\u0442\u0435\u0442 \u0421\u0442\u043e\u0443\u043d\u0438 \u0411\u0440\u0443\u043a, \u041d\u044c\u044e-\u0419\u043e\u0440\u043a<\/p>\n<p>&nbsp;<\/p>\n<p>\u041f\u043e\u0441\u0442\u0434\u043e\u043a\u0442\u043e\u0440\u0441\u043a\u0430\u044f \u0441\u0442\u0438\u043f\u0435\u043d\u0434\u0438\u044f, \u0423\u043d\u0438\u0432\u0435\u0440\u0441\u0438\u0442\u0435\u0442 \u0411\u0440\u0438\u0442\u0430\u043d\u0441\u043a\u043e\u0439 \u041a\u043e\u043b\u0443\u043c\u0431\u0438\u0438, \u0411\u0440\u0438\u0442\u0430\u043d\u0441\u043a\u0430\u044f \u041a\u043e\u043b\u0443\u043c\u0431\u0438\u044f, \u041a\u0430\u043d\u0430\u0434\u0430<\/p>\n<p>&nbsp;<\/p>\n<p><strong>\u0427\u0442\u043e \u043f\u0440\u0438\u0432\u0435\u043b\u043e \u0432\u0430\u0441 \u043a \u043a\u0430\u0440\u044c\u0435\u0440\u0435 \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u0442\u0435\u043b\u044f \u0438, \u0432 \u0447\u0430\u0441\u0442\u043d\u043e\u0441\u0442\u0438, \u043a \u0438\u0437\u0443\u0447\u0435\u043d\u0438\u044e \u043c\u044b\u0448\u0435\u0447\u043d\u043e\u0439 \u0434\u0438\u0441\u0442\u0440\u043e\u0444\u0438\u0438?<\/strong><\/p>\n<p>&nbsp;<\/p>\n<p>2 \u043f\u0440\u0438\u0447\u0438\u043d\u044b:<\/p>\n<p>&nbsp;<\/p>\n<p>\u042f \u0438\u0437\u0443\u0447\u0430\u044e \u043c\u043e\u043b\u0435\u043a\u0443\u043b\u044f\u0440\u043d\u044b\u0435 \u043d\u043e\u0436\u043d\u0438\u0446\u044b, \u043d\u0430\u0437\u044b\u0432\u0430\u0435\u043c\u044b\u0435 \u043f\u0440\u043e\u0442\u0435\u0430\u0437\u0430\u043c\u0438, \u043a\u043e\u0442\u043e\u0440\u044b\u0435 \u0432\u044b\u043f\u043e\u043b\u043d\u044f\u044e\u0442 \u043c\u043d\u043e\u0436\u0435\u0441\u0442\u0432\u043e \u0444\u0443\u043d\u043a\u0446\u0438\u0439 \u0432\u043e \u0432\u0441\u0435\u0445 \u0430\u0441\u043f\u0435\u043a\u0442\u0430\u0445 \u0436\u0438\u0437\u043d\u0438, \u0431\u0438\u043e\u043b\u043e\u0433\u0438\u0447\u0435\u0441\u043a\u0438\u0445 \u0444\u0443\u043d\u043a\u0446\u0438\u044f\u0445 \u0438 \u043f\u0440\u0438 \u0440\u0430\u0437\u043b\u0438\u0447\u043d\u044b\u0445 \u043c\u044b\u0448\u0435\u0447\u043d\u044b\u0445 \u0434\u0438\u0441\u0442\u0440\u043e\u0444\u0438\u044f\u0445. \u041c\u044b\u0448\u0435\u0447\u043d\u0430\u044f \u0434\u0438\u0441\u0442\u0440\u043e\u0444\u0438\u044f \u0442\u0438\u043f\u0430 2A (LGMD2A) \u0432\u044b\u0437\u044b\u0432\u0430\u0435\u0442\u0441\u044f \u043c\u0443\u0442\u0430\u0446\u0438\u0435\u0439 \u043e\u0434\u043d\u043e\u0439 \u0438\u0437 \u043f\u0440\u043e\u0442\u0435\u0430\u0437, \u043d\u0430\u0437\u044b\u0432\u0430\u0435\u043c\u043e\u0439 Calpain-3. \u042f \u0440\u0430\u0431\u043e\u0442\u0430\u044e \u043d\u0430\u0434 \u0442\u0435\u043c, \u0447\u0442\u043e\u0431\u044b \u043b\u0443\u0447\u0448\u0435 \u043f\u043e\u043d\u044f\u0442\u044c \u0435\u0433\u043e \u0444\u0443\u043d\u043a\u0446\u0438\u0438 \u0432 \u0431\u0438\u043e\u043b\u043e\u0433\u0438\u0438 \u043c\u044b\u0448\u0446.<\/p>\n<p>&nbsp;<\/p>\n<p>\u0423 \u043c\u043e\u0435\u0433\u043e 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\u0440\u0435\u0431\u0435\u043d\u043a\u043e\u043c\/\u043f\u043e\u0434\u0440\u043e\u0441\u0442\u043a\u043e\u043c (\u043e\u043d \u0441\u043a\u043e\u043d\u0447\u0430\u043b\u0441\u044f, \u043a\u043e\u0433\u0434\u0430 \u043c\u043d\u0435 \u0431\u044b\u043b\u043e 14 \u043b\u0435\u0442), \u043d\u043e \u0438\u043c\u0435\u043d\u043d\u043e \u044d\u0442\u043e \u043e\u043f\u0440\u0435\u0434\u0435\u043b\u0438\u043b\u043e \u043c\u043e\u0439 \u0432\u044b\u0431\u043e\u0440 \u043f\u0440\u043e\u0444\u0435\u0441\u0441\u0438\u0438. \u042f \u0432\u0441\u0435\u0433\u0434\u0430 \u0445\u043e\u0442\u0435\u043b \u0441\u0442\u0430\u0442\u044c \u0443\u0447\u0435\u043d\u044b\u043c, \u0447\u0442\u043e\u0431\u044b \u043e\u0445\u0430\u0440\u0430\u043a\u0442\u0435\u0440\u0438\u0437\u043e\u0432\u0430\u0442\u044c \u0434\u0435\u0444\u0435\u043a\u0442\u044b \u043c\u044b\u0448\u0435\u0447\u043d\u044b\u0445 \u0444\u0443\u043d\u043a\u0446\u0438\u0439 \u0443 \u0431\u043e\u043b\u044c\u043d\u044b\u0445 \u041c\u0414 \u0438 \u043b\u0443\u0447\u0448\u0435 \u043f\u043e\u043d\u044f\u0442\u044c, \u043a\u0430\u043a \u043f\u0440\u043e\u0442\u0435\u043a\u0430\u0435\u0442 \u044d\u0442\u0430 \u0431\u043e\u043b\u0435\u0437\u043d\u044c. \u041f\u043e\u0441\u043b\u0435 \u0434\u0435\u0441\u044f\u0442\u0438\u043b\u0435\u0442\u0438\u0439 \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0439 \u0434\u043e \u0441\u0438\u0445 \u043f\u043e\u0440 \u043e\u0441\u0442\u0430\u0435\u0442\u0441\u044f \u043c\u043d\u043e\u0433\u043e \u0437\u0430\u0433\u0430\u0434\u043e\u043a, \u0441\u0432\u044f\u0437\u0430\u043d\u043d\u044b\u0445 \u0441 \u041c\u0414, \u0438 \u044f \u043d\u0430\u0445\u043e\u0436\u0443 \u044d\u0442\u043e \u0443\u0432\u043b\u0435\u043a\u0430\u0442\u0435\u043b\u044c\u043d\u044b\u043c.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>\u041a\u0430\u043a\u0438\u0435 \u0442\u0435\u043c\u044b \u0432\u044b \u0438\u0437\u0443\u0447\u0430\u0435\u0442\u0435?<\/strong><\/p>\n<p>&nbsp;<\/p>\n<p>\u042f 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\u043e\u0431\u043d\u0430\u0440\u0443\u0436\u0438\u0432\u0430\u0442\u044c \u0438 \u043e\u0442\u0441\u043b\u0435\u0436\u0438\u0432\u0430\u0442\u044c \u0442\u044b\u0441\u044f\u0447\u0438 \u0431\u0435\u043b\u043a\u043e\u0432. \u042d\u0442\u043e \u043f\u043e\u0437\u0432\u043e\u043b\u044f\u0435\u0442 \u043d\u0430\u043c \u0440\u0430\u0441\u0441\u043c\u0430\u0442\u0440\u0438\u0432\u0430\u0442\u044c \u0441\u043e\u0441\u0442\u043e\u044f\u043d\u0438\u0435 \u0431\u043e\u043b\u0435\u0437\u043d\u0438 \u043d\u0430 \u0441\u0438\u0441\u0442\u0435\u043c\u043d\u043e\u043c \u0443\u0440\u043e\u0432\u043d\u0435 \u0438 \u043e\u0442\u0441\u043b\u0435\u0436\u0438\u0432\u0430\u0442\u044c \u043c\u043d\u043e\u0436\u0435\u0441\u0442\u0432\u043e \u0438\u0437\u043c\u0435\u043d\u0435\u043d\u0438\u0439 \u043e\u0434\u043d\u043e\u0432\u0440\u0435\u043c\u0435\u043d\u043d\u043e. \u041c\u044b \u043f\u0440\u0438\u043c\u0435\u043d\u044f\u0435\u043c \u043d\u0430\u0448\u0438 \u043c\u0435\u0442\u043e\u0434\u044b \u0434\u043b\u044f \u043e\u043f\u0440\u0435\u0434\u0435\u043b\u0435\u043d\u0438\u044f \u0445\u0430\u0440\u0430\u043a\u0442\u0435\u0440\u0438\u0441\u0442\u0438\u043a \u043a\u0430\u043b\u044c\u043f\u0430\u0438\u043d\u0430-3, \u0434\u0435\u0444\u0435\u043a\u0442 \u043a\u043e\u0442\u043e\u0440\u043e\u0433\u043e \u043d\u0430\u043f\u0440\u044f\u043c\u0443\u044e \u0432\u044b\u0437\u044b\u0432\u0430\u0435\u0442 LGMD2A.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>\u041a\u0430\u043a \u0432\u0430\u0448\u0430 \u0440\u0430\u0431\u043e\u0442\u0430 \u043f\u043e\u043c\u043e\u0436\u0435\u0442 \u043f\u0430\u0446\u0438\u0435\u043d\u0442\u0430\u043c? \u041e\u043d\u0430 \u043d\u043e\u0441\u0438\u0442 \u0431\u043e\u043b\u044c\u0448\u0435 \u043d\u0430\u0443\u0447\u043d\u044b\u0439 \u0445\u0430\u0440\u0430\u043a\u0442\u0435\u0440 \u0438\u043b\u0438 \u043c\u043e\u0436\u0435\u0442 \u0441\u0442\u0430\u0442\u044c \u043c\u0435\u0442\u043e\u0434\u043e\u043c \u043b\u0435\u0447\u0435\u043d\u0438\u044f LGMD \u0438\u043b\u0438 MD 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