{"id":648,"date":"2016-01-22T14:53:41","date_gmt":"2016-01-22T22:53:41","guid":{"rendered":"http:\/\/www.quintanalab.org\/?p=648"},"modified":"2016-01-22T14:53:41","modified_gmt":"2016-01-22T22:53:41","slug":"research-altered-protein-modifications-in-mitochondrial-disease","status":"publish","type":"post","link":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/2016\/01\/22\/research-altered-protein-modifications-in-mitochondrial-disease\/","title":{"rendered":"Research: Altered protein modifications in mitochondrial disease"},"content":{"rendered":"<p>Our latest paper is out!<\/p>\n<p>In collaboration with the <strong><a href=\"http:\/\/ppn.med.sc.edu\/nfrizzell.asp\" target=\"_blank\">Frizzell lab<\/a><\/strong> at the <strong><a href=\"http:\/\/www.med.sc.edu\/\" target=\"_blank\">University of South Carolina<\/a><\/strong>\u00a0we have published the article titled:<\/p>\n<blockquote><p><em><strong>\u00ab<a href=\"http:\/\/www.mcponline.org\/content\/early\/2015\/10\/08\/mcp.M115.051516.long\" target=\"_blank\">Succination is increased on select proteins in the brainstem of the Ndufs4 knockout mouse, a model of Leigh syndrome.<\/a>\u00bb\u00a0<\/strong><\/em><\/p><\/blockquote>\n<p>in the journal <strong>Molecular and Cellular Proteomics<\/strong>.<\/p>\n<p>In this paper, we have been able to identify that the<strong> metabolic deficits<\/strong> mediated by mitochondrial dysfunction (we used our Leigh Syndrome mice for this study) lead to <strong>permanent modifications<\/strong> in discrete proteins in affected neurons.<\/p>\n<p>These modifications (called <strong>succination<\/strong>) have been shown to impair the activity of proteins in other studies, therefore we believe these mitochondrial proteins will also <strong>lose function<\/strong> in our model. Interestingly, our study shows that these modifications are only observed in cells residing in areas affected by the pathology, further enhancing the idea that they may mediate the<strong> selective damage observed in\u00a0mitochondrial disease<\/strong>.<\/p>\n<p>The main\u00a0proteins observed to be altered, <strong>VDAC1<\/strong> and <strong>VDAC2<\/strong>, are key factors controlling <strong>transport of ions and molecules inside the mitochondria<\/strong>, so these results open a new and interesting line of research in the lab in the overarching goal of finding a cure for mito disease.<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Our latest paper is out! In collaboration with the Frizzell lab at the University of South Carolina\u00a0we have published the article titled: \u00abSuccination is increased on select proteins in the brainstem of the Ndufs4 knockout mouse, a model of Leigh syndrome.\u00bb\u00a0 in the journal Molecular and Cellular Proteomics. In this paper, we have been able [&hellip;]<\/p>\n","protected":false},"author":2072,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[7,13,14],"tags":[],"class_list":["post-648","post","type-post","status-publish","format-standard","hentry","category-albert","category-articles","category-research"],"_links":{"self":[{"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/posts\/648","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/users\/2072"}],"replies":[{"embeddable":true,"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/comments?post=648"}],"version-history":[{"count":0,"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/posts\/648\/revisions"}],"wp:attachment":[{"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/media?parent=648"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/categories?post=648"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/webs.uab.cat\/mitochondrial-neuropatology\/wp-json\/wp\/v2\/tags?post=648"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}