{"id":519,"date":"2013-04-09T18:34:06","date_gmt":"2013-04-09T18:34:06","guid":{"rendered":"http:\/\/stg-blogs.bmj.com\/jmg\/?p=519"},"modified":"2013-04-09T18:34:06","modified_gmt":"2013-04-09T18:34:06","slug":"troponin-activator-augments-muscle-force-in-nemaline-myopathy-patients-with-nebulin-mutations","status":"publish","type":"post","link":"https:\/\/stg-blogs.bmj.com\/jmg\/2013\/04\/09\/troponin-activator-augments-muscle-force-in-nemaline-myopathy-patients-with-nebulin-mutations\/","title":{"rendered":"Troponin activator augments muscle force in nemaline myopathy patients with nebulin mutations"},"content":{"rendered":"<p>Nemaline myopathy (NM) is the most common non-dystrophic congenital myopathy and is most frequently caused by mutations in the nebulin gene. The sarcomeric protein nebulin plays a crucial role in skeletal muscle performance. NM patients with nebulin mutations have muscle weakness through mechanisms including a lower responsiveness to calcium. No therapy exists to treat muscle weakness in NM. Here, we show that the novel fast skeletal muscle troponin activator, CK-2066260, augments force generation at submaximal calcium levels &#8211; levels that are typically used for daily life activities. Thus, fast troponin activation is a therapeutic mechanism to augment muscle strength in NM patients with nebulin mutations. (By Dr. Josine de Winter, <a href=\"http:\/\/jmg.bmj.com\/content\/early\/2013\/04\/08\/jmedgenet-2012-101470\">http:\/\/jmg.bmj.com\/content\/early\/2013\/04\/08\/jmedgenet-2012-101470<\/a> )<!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Nemaline myopathy (NM) is the most common non-dystrophic congenital myopathy and is most frequently caused by mutations in the nebulin gene. The sarcomeric protein nebulin plays a crucial role in skeletal muscle performance. NM patients with nebulin mutations have muscle weakness through mechanisms including a lower responsiveness to calcium. No therapy exists to treat muscle [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/stg-blogs.bmj.com\/jmg\/2013\/04\/09\/troponin-activator-augments-muscle-force-in-nemaline-myopathy-patients-with-nebulin-mutations\/\">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-519","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\/519","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=519"}],"version-history":[{"count":0,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/posts\/519\/revisions"}],"wp:attachment":[{"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/media?parent=519"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/categories?post=519"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/stg-blogs.bmj.com\/jmg\/wp-json\/wp\/v2\/tags?post=519"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}