{"id":3178,"date":"2020-01-12T12:52:48","date_gmt":"2020-01-12T18:52:48","guid":{"rendered":"https:\/\/restruct-lgmd-2021.pantheonsite.io\/?p=3178"},"modified":"2020-01-12T12:52:48","modified_gmt":"2020-01-12T18:52:48","slug":"natasha","status":"publish","type":"post","link":"https:\/\/www.lgmd-info.org\/ru\/individuals-with-lgmd-interviews\/2020\/01\/12\/natasha\/","title":{"rendered":"\u0418\u041d\u0414\u0418\u0412\u0418\u0414\u0423\u0410\u041b \u0421 \u041b\u0413\u041c\u0414: \u041d\u0430\u0442\u0430\u0448\u0430"},"content":{"rendered":"
\"\"<\/figure>\n\n\n\n

LGMD \"\u0418\u041d\u0422\u0415\u0420\u0412\u042c\u042e \u0412 \u0426\u0415\u041d\u0422\u0420\u0415 \u0412\u041d\u0418\u041c\u0410\u041d\u0418\u042f\"<\/h3>\n\n\n\n

\u0418\u043c\u044f<\/strong>: \u041d\u0430\u0442\u0430\u0448\u0430 \n\u0412\u043e\u0437\u0440\u0430\u0441\u0442<\/strong>:\n25 \u043b\u0435\u0442<\/p>\n\n\n\n

\u0421\u0442\u0440\u0430\u043d\u0430<\/strong>: \u0421\u043e\u0435\u0434\u0438\u043d\u0435\u043d\u043d\u044b\u0435 \u0428\u0442\u0430\u0442\u044b<\/p>\n\n\n\n

LGMD \u041f\u043e\u0434\u0442\u0438\u043f<\/strong>: LGMD D1, \u0441\u0432\u044f\u0437\u0430\u043d\u043d\u044b\u0439 \u0441 DNAJB6 (\u0440\u0430\u043d\u0435\u0435 LGMD \u0442\u0438\u043f\n1D)<\/p>\n\n\n\n

\u0412 \u043a\u0430\u043a\u043e\u043c \u0432\u043e\u0437\u0440\u0430\u0441\u0442\u0435 \u0432\u0430\u043c \u0431\u044b\u043b \u043f\u043e\u0441\u0442\u0430\u0432\u043b\u0435\u043d \u0434\u0438\u0430\u0433\u043d\u043e\u0437<\/strong>:<\/p>\n\n\n\n

\u0414\u0438\u0430\u0433\u043d\u043e\u0437 \u043c\u043d\u0435 \u043f\u043e\u0441\u0442\u0430\u0432\u0438\u043b\u0438 \u0432 \u0432\u043e\u0437\u0440\u0430\u0441\u0442\u0435 23 \u043b\u0435\u0442.<\/p>\n\n\n\n

\u041a\u0430\u043a\u043e\u0432\u044b \u0431\u044b\u043b\u0438 \u0432\u0430\u0448\u0438 \u043f\u0435\u0440\u0432\u044b\u0435 \u0441\u0438\u043c\u043f\u0442\u043e\u043c\u044b<\/strong>:<\/p>\n\n\n\n

\u041d\u0430\u0448 \u043f\u043e\u0434\u0442\u0438\u043f - \u043f\u043e\u0437\u0434\u043d\u0438\u0439, \u043f\u043e\u044d\u0442\u043e\u043c\u0443 \u0432 \u043d\u0430\u0441\u0442\u043e\u044f\u0449\u0435\u0435 \u0432\u0440\u0435\u043c\u044f \u0443 \u043c\u0435\u043d\u044f \u043d\u0435\u0442 \u043d\u0438\u043a\u0430\u043a\u0438\u0445 \u0441\u0438\u043c\u043f\u0442\u043e\u043c\u043e\u0432, \u0445\u043e\u0442\u044f \u0432 \u0434\u0435\u0442\u0441\u0442\u0432\u0435 \u0443 \u043c\u0435\u043d\u044f \u0431\u044b\u043b\u0438 \u043d\u0435\u0431\u043e\u043b\u044c\u0448\u0438\u0435 \u043f\u0440\u043e\u0431\u043b\u0435\u043c\u044b, \u043a\u043e\u0442\u043e\u0440\u044b\u0435 \u0442\u0435\u043f\u0435\u0440\u044c, \u043a\u043e\u0433\u0434\u0430 \u044f \u0437\u043d\u0430\u044e \u043e \u0441\u0432\u043e\u0435\u043c \u0434\u0438\u0430\u0433\u043d\u043e\u0437\u0435, \u0441\u0442\u0430\u043b\u0438 \u0431\u043e\u043b\u0435\u0435 \u043f\u043e\u043d\u044f\u0442\u043d\u044b\u043c\u0438. \u0412 \u0434\u0435\u0442\u0441\u0442\u0432\u0435 \u044f \u043d\u0435 \u043c\u043e\u0433\u043b\u0430 \u0431\u0435\u0433\u0430\u0442\u044c \u0442\u0430\u043a \u0436\u0435 \u0431\u044b\u0441\u0442\u0440\u043e, \u043a\u0430\u043a \u043c\u043e\u0438 \u0441\u0432\u0435\u0440\u0441\u0442\u043d\u0438\u043a\u0438, \u0430 \u0432 \u0442\u0430\u043d\u0446\u0430\u0445 \u043c\u043d\u0435 \u0431\u044b\u043b\u043e \u0442\u0440\u0443\u0434\u043d\u043e \u043f\u043e\u0434\u043d\u0438\u043c\u0430\u0442\u044c\u0441\u044f \u0441 \u0437\u0435\u043c\u043b\u0438, \u043d\u0435 \u0438\u0441\u043f\u043e\u043b\u044c\u0437\u0443\u044f \u0440\u0443\u043a\u0438, \u043d\u0430\u043f\u0440\u0438\u043c\u0435\u0440.<\/p>\n\n\n\n

\u0415\u0441\u0442\u044c \u043b\u0438 \u0443 \u0432\u0430\u0441 \u0434\u0440\u0443\u0433\u0438\u0435 \u0447\u043b\u0435\u043d\u044b \u0441\u0435\u043c\u044c\u0438, \u0441\u0442\u0440\u0430\u0434\u0430\u044e\u0449\u0438\u0435 LGMD:<\/strong><\/p>\n\n\n\n

\u0414\u0430, \u0443 \u043c\u043e\u0435\u0433\u043e \u043e\u0442\u0446\u0430, \u0434\u044f\u0434\u0438 (\u043f\u043e \u043e\u0442\u0446\u043e\u0432\u0441\u043a\u043e\u0439 \u043b\u0438\u043d\u0438\u0438) \u0438 \u0434\u0435\u0434\u0430 \u043f\u043e \u043e\u0442\u0446\u043e\u0432\u0441\u043a\u043e\u0439 \u043b\u0438\u043d\u0438\u0438\n\u0434\u0435\u0434\u0443\u0448\u043a\u0430 \u0441 \u0442\u0430\u043a\u0438\u043c \u0436\u0435 \u0434\u0438\u0430\u0433\u043d\u043e\u0437\u043e\u043c.<\/p>\n\n\n\n

\u0427\u0442\u043e, \u043f\u043e \u0432\u0430\u0448\u0435\u043c\u0443 \u043c\u043d\u0435\u043d\u0438\u044e, \u043f\u0440\u0435\u0434\u0441\u0442\u0430\u0432\u043b\u044f\u0435\u0442 \u043d\u0430\u0438\u0431\u043e\u043b\u044c\u0448\u0443\u044e \u0442\u0440\u0443\u0434\u043d\u043e\u0441\u0442\u044c\n\u0432 \u0436\u0438\u0437\u043d\u0438 \u0441 LGMD<\/strong>:<\/p>\n\n\n\n

\u0421\u0430\u043c\u044b\u043c \u0431\u043e\u043b\u044c\u0448\u0438\u043c \u0438\u0441\u043f\u044b\u0442\u0430\u043d\u0438\u0435\u043c \u0434\u043b\u044f \u043c\u0435\u043d\u044f \u0431\u044b\u043b\u043e \u043e\u0441\u043e\u0437\u043d\u0430\u043d\u0438\u0435 \u0442\u043e\u0433\u043e, \u0447\u0442\u043e \u0432 \u043e\u0434\u0438\u043d \u043f\u0440\u0435\u043a\u0440\u0430\u0441\u043d\u044b\u0439 \u0434\u0435\u043d\u044c\n\u043c\u043e\u044f \u043f\u043e\u0434\u0432\u0438\u0436\u043d\u043e\u0441\u0442\u044c \u0441\u0442\u0430\u043d\u0435\u0442 \u043c\u0435\u043d\u044c\u0448\u0435, \u0447\u0435\u043c \u0441\u0435\u0439\u0447\u0430\u0441, \u0438 \u044f \u043d\u0435 \u0441\u043c\u043e\u0433\u0443 \u043f\u0440\u043e\u0434\u043e\u043b\u0436\u0430\u0442\u044c\n\u0432\u0435\u0441\u0442\u0438 \u0430\u043a\u0442\u0438\u0432\u043d\u044b\u0439 \u043e\u0431\u0440\u0430\u0437 \u0436\u0438\u0437\u043d\u0438, \u043a\u043e\u0442\u043e\u0440\u044b\u0439 \u043c\u043d\u0435 \u0441\u0435\u0439\u0447\u0430\u0441 \u043d\u0440\u0430\u0432\u0438\u0442\u0441\u044f.<\/p>\n\n\n\n

\u041a\u0430\u043a\u043e\u0435 \u0432\u0430\u0448\u0435 \u0441\u0430\u043c\u043e\u0435 \u0431\u043e\u043b\u044c\u0448\u043e\u0435 \u0434\u043e\u0441\u0442\u0438\u0436\u0435\u043d\u0438\u0435<\/strong>:<\/p>\n\n\n\n

\u042f \u043d\u0435 \u0443\u0432\u0435\u0440\u0435\u043d, \u0447\u0442\u043e \u043c\u043e\u0433\u0443 \u043d\u0430\u0437\u0432\u0430\u0442\u044c \u0441\u0432\u043e\u0435 \u0441\u0430\u043c\u043e\u0435 \u0431\u043e\u043b\u044c\u0448\u043e\u0435 \u0434\u043e\u0441\u0442\u0438\u0436\u0435\u043d\u0438\u0435... \u042f \u0431\u044b \u0441\u043a\u0430\u0437\u0430\u043b, \u0447\u0442\u043e \u044d\u0442\u043e \u0441\u0435\u043c\u0435\u0439\u043d\u044b\u0435 \u0438 \u0434\u0440\u0443\u0436\u0435\u0441\u043a\u0438\u0435 \u0441\u0432\u044f\u0437\u0438, \u043a\u043e\u0442\u043e\u0440\u044b\u0435 \u044f \u0441\u043e\u0445\u0440\u0430\u043d\u0438\u043b. \u041f\u043b\u044e\u0441 \u0441\u043e\u043e\u0431\u0449\u0435\u0441\u0442\u0432\u043e, \u043a\u043e\u0442\u043e\u0440\u043e\u0435 \u043c\u044b \u0441\u043e\u0437\u0434\u0430\u043b\u0438 \u0438 \u0441\u043e\u0437\u0434\u0430\u0435\u043c \u0434\u043b\u044f \u0434\u043e\u043c\u0438\u043d\u0430\u043d\u0442\u043d\u044b\u0445 \u0444\u043e\u0440\u043c LGMD \u0447\u0435\u0440\u0435\u0437 \u0424\u043e\u043d\u0434 LGMD-1D DNAJB6, \u043a\u043e\u0442\u043e\u0440\u044b\u0439 \u044f\u0432\u043b\u044f\u0435\u0442\u0441\u044f \u043d\u0435\u043a\u043e\u043c\u043c\u0435\u0440\u0447\u0435\u0441\u043a\u0438\u043c \u043e\u0431\u0449\u0435\u0441\u0442\u0432\u0435\u043d\u043d\u044b\u043c \u0444\u043e\u043d\u0434\u043e\u043c, \u0447\u044c\u044f \u043c\u0438\u0441\u0441\u0438\u044f \u0441\u043e\u0441\u0442\u043e\u0438\u0442 \u0432 \u0442\u043e\u043c, \u0447\u0442\u043e\u0431\u044b \u043f\u043e\u0434\u0434\u0435\u0440\u0436\u0438\u0432\u0430\u0442\u044c \u0446\u0435\u043d\u0442\u0440\u0430\u043b\u044c\u043d\u043e\u0435 \u043c\u0435\u0441\u0442\u043e \u0434\u043b\u044f \u043b\u044e\u0434\u0435\u0439 \u0441 \u0434\u0438\u0430\u0433\u043d\u043e\u0437\u043e\u043c \u043c\u044b\u0448\u0435\u0447\u043d\u043e\u0439 \u0434\u0438\u0441\u0442\u0440\u043e\u0444\u0438\u0438 \u043a\u043e\u043d\u0435\u0447\u043d\u043e\u0441\u0442\u0435\u0439 \u0442\u0438\u043f\u0430 1D (LGMD1D), \u0447\u0442\u043e\u0431\u044b \u0432\u0441\u0442\u0440\u0435\u0447\u0430\u0442\u044c\u0441\u044f, \u043e\u0431\u044a\u0435\u0434\u0438\u043d\u044f\u0442\u044c \u0432\u0430\u0436\u043d\u044b\u0435 \u043d\u043e\u0432\u043e\u0441\u0442\u0438 \u0438 \u043d\u0430\u0447\u0438\u043d\u0430\u0442\u044c \u0443\u0441\u0438\u043b\u0438\u044f \u043f\u043e \u0441\u0431\u043e\u0440\u0443 \u0441\u0440\u0435\u0434\u0441\u0442\u0432 \u0434\u043b\u044f \u043f\u0440\u043e\u0434\u0432\u0438\u0436\u0435\u043d\u0438\u044f \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0439.  https:\/\/lgmd1d.org<\/a>  \u041c\u044b \u0442\u0430\u043a\u0436\u0435 \u0441\u043e\u0437\u0434\u0430\u043b\u0438 \u0440\u0435\u0435\u0441\u0442\u0440 \u043f\u0430\u0446\u0438\u0435\u043d\u0442\u043e\u0432 \u0434\u043b\u044f \u0432\u0441\u0435\u0445 \u043f\u0430\u0446\u0438\u0435\u043d\u0442\u043e\u0432 \u0441 \u0434\u0438\u0430\u0433\u043d\u043e\u0437\u043e\u043c \u0430\u0443\u0442\u043e\u0441\u043e\u043c\u043d\u043e-\u0434\u043e\u043c\u0438\u043d\u0430\u043d\u0442\u043d\u043e\u0439 \u0444\u043e\u0440\u043c\u044b LGMD.  <\/p>\n\n\n\n

\u041a\u0430\u043a LGMD \u043f\u043e\u0432\u043b\u0438\u044f\u043b \u043d\u0430 \u0432\u0430\u0441, \u0447\u0442\u043e\u0431\u044b \u0432\u044b \u0441\u0442\u0430\u043b\u0438 \u0442\u0435\u043c.\n\u041a\u0430\u043a \u041b\u0413\u041c\u0414 \u043f\u043e\u0432\u043b\u0438\u044f\u043b \u043d\u0430 \u0442\u043e, \u0447\u0442\u043e\u0431\u044b \u0432\u044b \u0441\u0442\u0430\u043b\u0438 \u0447\u0435\u043b\u043e\u0432\u0435\u043a\u043e\u043c, \u043a\u043e\u0442\u043e\u0440\u044b\u043c \u0432\u044b \u044f\u0432\u043b\u044f\u0435\u0442\u0435\u0441\u044c \u0441\u0435\u0433\u043e\u0434\u043d\u044f:<\/strong><\/p>\n\n\n\n

\u042f \u043f\u043e\u0441\u0442\u043e\u044f\u043d\u043d\u043e \u0434\u0443\u043c\u0430\u044e \u043e \u0431\u0443\u0434\u0443\u0449\u0435\u043c, \u043e \u0442\u043e\u043c, \u043a\u0430\u043a \u043c\u043e\u0438 \u0434\u0435\u0439\u0441\u0442\u0432\u0438\u044f\n\u043f\u043e\u0432\u043b\u0438\u044f\u044e\u0442 \u043d\u0430 \u043c\u043e\u0435 \u0442\u0435\u043b\u043e, \u043b\u044e\u0434\u0435\u0439, \u043a\u043e\u0442\u043e\u0440\u044b\u0445 \u044f \u043b\u044e\u0431\u043b\u044e, \u0438 \u0441\u0435\u043c\u044c\u044e, \u043a\u043e\u0442\u043e\u0440\u0443\u044e \u044f \u043d\u0430\u0434\u0435\u044e\u0441\u044c \u0432\u044b\u0440\u0430\u0441\u0442\u0438\u0442\u044c. \u042d\u0442\u043e \u0437\u0430\u0441\u0442\u0430\u0432\u0438\u043b\u043e \u043c\u0435\u043d\u044f \u043d\u0430\u0431\u0440\u0430\u0442\u044c\u0441\u044f \u0442\u0435\u0440\u043f\u0435\u043d\u0438\u044f \u0438 \u043f\u043e\u0441\u0442\u0430\u0440\u0430\u0442\u044c\u0441\u044f\n\u0432\u0435\u0441\u0442\u0438 \u043c\u0435\u043d\u0435\u0435 \u0444\u0438\u0437\u0438\u0447\u0435\u0441\u043a\u0438 \u043d\u0430\u043f\u0440\u044f\u0436\u0435\u043d\u043d\u044b\u0439 \u043e\u0431\u0440\u0430\u0437 \u0436\u0438\u0437\u043d\u0438.<\/p>\n\n\n\n

\u0427\u0442\u043e \u0432\u044b \u0445\u043e\u0442\u0438\u0442\u0435, \u0447\u0442\u043e\u0431\u044b \u043c\u0438\u0440 \u0443\u0437\u043d\u0430\u043b \u043e LGMD<\/strong>:<\/p>\n\n\n\n

\u041c\u044b \u0434\u043e\u043b\u0436\u043d\u044b \u043f\u0440\u043e\u0434\u043e\u043b\u0436\u0430\u0442\u044c\n\u0440\u0430\u0441\u043f\u0440\u043e\u0441\u0442\u0440\u0430\u043d\u044f\u0442\u044c \u0438\u043d\u0444\u043e\u0440\u043c\u0430\u0446\u0438\u044e \u0438 \u043d\u0430\u043b\u0430\u0436\u0438\u0432\u0430\u0442\u044c \u0441\u0432\u044f\u0437\u0438, \u0441\u043f\u043e\u0441\u043e\u0431\u0441\u0442\u0432\u0443\u044e\u0449\u0438\u0435 \u0438\u0437\u043b\u0435\u0447\u0435\u043d\u0438\u044e. \u041d\u0430\u043f\u0440\u0438\u043c\u0435\u0440, \u0440\u0435\u0435\u0441\u0442\u0440 \u043f\u0430\u0446\u0438\u0435\u043d\u0442\u043e\u0432 \u0441 \u0430\u0443\u0442\u043e\u0441\u043e\u043c\u043d\u043e-\u0434\u043e\u043c\u0438\u043d\u0430\u043d\u0442\u043d\u044b\u043c \u041b\u0413\u041c\u0414\n(LGMD \u0442\u0438\u043f\u0430 1) \u0431\u0443\u0434\u0435\u0442 \u0438\u043c\u0435\u0442\u044c \u0436\u0438\u0437\u043d\u0435\u043d\u043d\u043e \u0432\u0430\u0436\u043d\u043e\u0435 \u0437\u043d\u0430\u0447\u0435\u043d\u0438\u0435 \u0434\u043b\u044f \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0439 \u0438 \u043f\u043e\u0438\u0441\u043a\u0430 \u043b\u0435\u0447\u0435\u043d\u0438\u044f.<\/p>\n\n\n\n

\u0415\u0441\u043b\u0438 \u0431\u044b \u0432\u0430\u0448\u0435 \u0437\u0430\u0431\u043e\u043b\u0435\u0432\u0430\u043d\u0438\u0435 LGMD \u043c\u043e\u0436\u043d\u043e \u0431\u044b\u043b\u043e \"\u0432\u044b\u043b\u0435\u0447\u0438\u0442\u044c\" \u0437\u0430\u0432\u0442\u0440\u0430, \u0447\u0442\u043e\n\u0447\u0442\u043e \u0431\u044b \u0432\u044b \u0445\u043e\u0442\u0435\u043b\u0438 \u0441\u0434\u0435\u043b\u0430\u0442\u044c \u0432 \u043f\u0435\u0440\u0432\u0443\u044e \u043e\u0447\u0435\u0440\u0435\u0434\u044c<\/strong>:<\/p>\n\n\n\n

\u0415\u0441\u043b\u0438 \u0431\u044b \u043c\u0435\u043d\u044f \u0432\u044b\u043b\u0435\u0447\u0438\u043b\u0438 \u0437\u0430\u0432\u0442\u0440\u0430, \u044f \u0431\u044b \u043f\u043e\u0448\u043b\u0430 \u0442\u0430\u043d\u0446\u0435\u0432\u0430\u0442\u044c, \u0447\u0442\u043e\u0431\u044b \u043e\u0442\u043f\u0440\u0430\u0437\u0434\u043d\u043e\u0432\u0430\u0442\u044c! <\/p>\n\n\n\n

\n\n\n\n

\u0427\u0442\u043e\u0431\u044b \u043f\u0440\u043e\u0447\u0438\u0442\u0430\u0442\u044c \u0434\u0440\u0443\u0433\u0438\u0435 \u0438\u043d\u0442\u0435\u0440\u0432\u044c\u044e \"LGMD Spotlight Interviews\" \u0438\u043b\u0438 \u0441\u0442\u0430\u0442\u044c \u0434\u043e\u0431\u0440\u043e\u0432\u043e\u043b\u044c\u0446\u0435\u043c \u0434\u043b\u044f \u0443\u0447\u0430\u0441\u0442\u0438\u044f \u0432 \u043f\u0440\u0435\u0434\u0441\u0442\u043e\u044f\u0449\u0435\u043c \u0438\u043d\u0442\u0435\u0440\u0432\u044c\u044e, \u043f\u043e\u0441\u0435\u0442\u0438\u0442\u0435 \u043d\u0430\u0448 \u0441\u0430\u0439\u0442: https:\/\/www.lgmd-info.org\/spotlight-interviews<\/a><\/p>","protected":false},"excerpt":{"rendered":"

LGMD “SPOTLIGHT INTERVIEW” Name:  Natasha  Age: 25 years old Country: […]<\/p>","protected":false},"author":1,"featured_media":3179,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[13,102,104],"tags":[],"class_list":["post-3178","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-individuals-with-lgmd-interviews","category-lgmd-d1-dnajb6-related","category-lgmd1d"],"acf":[],"yoast_head":"\nINDIVIDUAL WITH LGMD: Natasha - 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=\"http:\/\/www.lgmd-info.org\/ru\/individuals-with-lgmd-interviews\/2020\/01\/12\/natasha\/\" \/>\n<meta property=\"og:locale\" content=\"ru_RU\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"INDIVIDUAL WITH LGMD: Natasha - 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