{"id":2486,"date":"2016-10-13T14:50:00","date_gmt":"2016-10-13T12:50:00","guid":{"rendered":"https:\/\/www.progettomitofusina2.com\/contributo-delle-nostre-ricercatrici-al-cmtr-consortium\/"},"modified":"2022-06-13T10:12:31","modified_gmt":"2022-06-13T08:12:31","slug":"the-contribution-of-our-researchers-to-cmtr-consortium","status":"publish","type":"post","link":"https:\/\/www.progettomitofusina2.com\/en\/the-contribution-of-our-researchers-to-cmtr-consortium\/","title":{"rendered":"The contribution of our researchers to CMTR Consortium"},"content":{"rendered":"\n<style type=\"text\/css\" data-created_by=\"avia_inline_auto\" id=\"style-css-av-l2opzghe-4044c0f47fe1087b60c76fd66045e2e1\">\n.avia-image-container.av-l2opzghe-4044c0f47fe1087b60c76fd66045e2e1 img.avia_image{\nbox-shadow:none;\n}\n.avia-image-container.av-l2opzghe-4044c0f47fe1087b60c76fd66045e2e1 .av-image-caption-overlay-center{\ncolor:#ffffff;\n}\n<\/style>\n<div  class='avia-image-container av-l2opzghe-4044c0f47fe1087b60c76fd66045e2e1 av-styling- avia-align-center  avia-builder-el-0  el_before_av_heading  avia-builder-el-first '   itemprop=\"image\" itemscope=\"itemscope\" itemtype=\"https:\/\/schema.org\/ImageObject\" ><div class=\"avia-image-container-inner\"><div class=\"avia-image-overlay-wrap\"><img decoding=\"async\" fetchpriority=\"high\" class='wp-image-1786 avia-img-lazy-loading-not-1786 avia_image ' src=\"https:\/\/www.progettomitofusina2.com\/wp-content\/uploads\/2022\/05\/1500x630-News_65.jpg\" alt='' title='1500x630-News_65'  height=\"630\" width=\"1500\"  itemprop=\"thumbnailUrl\" srcset=\"https:\/\/www.progettomitofusina2.com\/wp-content\/uploads\/2022\/05\/1500x630-News_65.jpg 1500w, https:\/\/www.progettomitofusina2.com\/wp-content\/uploads\/2022\/05\/1500x630-News_65-300x126.jpg 300w, https:\/\/www.progettomitofusina2.com\/wp-content\/uploads\/2022\/05\/1500x630-News_65-1030x433.jpg 1030w, https:\/\/www.progettomitofusina2.com\/wp-content\/uploads\/2022\/05\/1500x630-News_65-768x323.jpg 768w, https:\/\/www.progettomitofusina2.com\/wp-content\/uploads\/2022\/05\/1500x630-News_65-705x296.jpg 705w\" sizes=\"(max-width: 1500px) 100vw, 1500px\" \/><\/div><\/div><\/div>\n\n<style type=\"text\/css\" data-created_by=\"avia_inline_auto\" id=\"style-css-av-l2opxhdy-8d4578e84ab72c3063958e234778cafc\">\n#top .av-special-heading.av-l2opxhdy-8d4578e84ab72c3063958e234778cafc{\npadding-bottom:10px;\n}\nbody .av-special-heading.av-l2opxhdy-8d4578e84ab72c3063958e234778cafc .av-special-heading-tag .heading-char{\nfont-size:25px;\n}\n.av-special-heading.av-l2opxhdy-8d4578e84ab72c3063958e234778cafc .av-subheading{\nfont-size:15px;\n}\n<\/style>\n<div  class='av-special-heading av-l2opxhdy-8d4578e84ab72c3063958e234778cafc av-special-heading-h2 blockquote modern-quote  avia-builder-el-1  el_after_av_image  el_before_av_textblock '><h2 class='av-special-heading-tag '  itemprop=\"headline\"  >The contribution of our researchers to CMTR Consortium<\/h2><div class='av-subheading av-subheading_below'><p>13 October 2016<\/p>\n<\/div><div class=\"special-heading-border\"><div class=\"special-heading-inner-border\"><\/div><\/div><\/div>\n<section  class='av_textblock_section av-l2opyaob-cf7f4c8d9bac44ea5d146e6b63ebea6b '   itemscope=\"itemscope\" itemtype=\"https:\/\/schema.org\/BlogPosting\" itemprop=\"blogPost\" ><div class='avia_textblock'  itemprop=\"text\" ><p>We publish an abstract of the study presented at the CMTR Consortium by our researchers<strong>\u00a0Dr\u00a0Federica Rizzo<\/strong>\u00a0and<strong>\u00a0Dr Agnese Ramirez<\/strong>\u00a0from Dino Ferrari Center:<\/p>\n<p><strong>FUNCTIONAL ANALYSIS AND GENOME-WIDE RNA-SEQ OF HUMAN MOTOR NEURONS IMPLICATE SELECTIVE MITOCHONDRIAL DEPLETION, RESISTANCE TO APOPTOSIS AND INCREASED MITOPHAGY IN CHARCOT-MARIE-TOOTH 2A<\/strong><\/p>\n<p>Rizzo F, Ramirez A., Ronchi R., Salani S, Nizzardo M, Fortunato F, Bordoni A, Bresolin N, Comi GP, Corti S.<br \/>\nDino Ferrari Centre, Neuroscience Section, Department of Pathophysiology and Transplantation(DEPT), Univerity of Milan, Neurology Unit, IRCCS Fondation C\u00e0 Granda Ospedale Maggiore Policlinico, Milan, Italy<\/p>\n<p>The disease type 2A Charcot-Marie-Tooth (CMT2A) is a sensorimotor polyneuropathy, characterized by the death of motor neurons and sensory, which results in progressive limb weakness, muscle atrophy and loss of sensory. The disease is due to mutations in the gene Mitofusina2 (MFN2), which encodes a protein localized at the mitochondria, organelles very important for the survival of the cells and their proper functioning. To date, unfortunately, there are no conclusive therapies for this condition.<br \/>\nThe reprogramming of these mature cells in induced pluripotent stem cells (iPSC) offers the possibility of obtaining patient-specific cells, such as human motor neurons and sensory neurons, typically affected in the disease, but impossible to obtain directly from patients with other strategies. In the study published online August 9, 2016, on Human Molecular Genetics, the research team of the Centro Dino Ferrari, University of Milan, Fondazione IRCCS Ca &#8216;Granda Ospedale Maggiore Policlinico , differentiated motor neurons (MN) from iPSCs derived from biopsy skin of patients with CMT2A. In this way, we have generated an in vitro model of the disease, currently not available. The cells thus obtained showed some typical aspects of the disease, such as reducing the amount of mitochondria and changes in their location, with no significant differences in survival. These defects are most apparent in neuronal cells results compared to skin fibroblasts, in agreement with the neuronal specificity of the disease. Overall, these data suggest that the reduction of mitochondria in motor neurons that express the mutated form of MFN2 , is not the result of a reduced production of mitochondria, but more likely the result of the death of these organelles. These mechanisms represent possible new molecular therapeutic targets for the development of an effective treatment for this disease.<\/p>\n<\/div><\/section>\n","protected":false},"excerpt":{"rendered":"<p>13 October 2016<br \/>\nWe publish an abstract of the study presented at the CMTR Consortium&#8230;<\/p>\n","protected":false},"author":1,"featured_media":1786,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[69,63],"tags":[],"class_list":["post-2486","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-2016-en","category-news-and-events"],"_links":{"self":[{"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/posts\/2486","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/comments?post=2486"}],"version-history":[{"count":5,"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/posts\/2486\/revisions"}],"predecessor-version":[{"id":2492,"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/posts\/2486\/revisions\/2492"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/media\/1786"}],"wp:attachment":[{"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/media?parent=2486"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/categories?post=2486"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.progettomitofusina2.com\/en\/wp-json\/wp\/v2\/tags?post=2486"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}