{"id":5416,"date":"2017-04-21T00:00:00","date_gmt":"2017-04-20T22:00:00","guid":{"rendered":"https:\/\/idibell.cat\/en\/blog\/2020\/05\/01\/researchers-describe-a-new-pathophysiological-process-of-megalencephalic-leukoencephalopathy-mlc-in-astrocytes\/"},"modified":"2020-05-18T11:01:31","modified_gmt":"2020-05-18T09:01:31","slug":"researchers-describe-a-new-pathophysiological-process-of-megalencephalic-leukoencephalopathy-mlc-in-astrocytes","status":"publish","type":"post","link":"https:\/\/idibell.cat\/en\/2017\/04\/researchers-describe-a-new-pathophysiological-process-of-megalencephalic-leukoencephalopathy-mlc-in-astrocytes\/","title":{"rendered":"Researchers describe a new pathophysiological process of megalencephalic leukoencephalopathy (MLC) in astrocytes"},"content":{"rendered":"<p>This paper, published in <em>Human Molecular Genetics<\/em>, addresses the pathophysiology of megalencephalic leukoencephalopathy (MLC) in astrocytes. Its main authors are Sonia Sirisi, Xabier Elorza-Vidal and Tanit Arnedo \u2013 affiliated to UB-IDIBELL and CIBERER. The work was co-directed by Xavier Gasull (Institute of Neurosciences-UB and IDIBAPS) and Ra\u00fal Est\u00e9vez (Institute of Neurosciences-UB, IDIBELL and CIBERER), in collaboration with Virginia Nunes (UB, IDIBELL and CIBERER). T<\/p>\n<p>he study concluded that ClC-2 (chloride channel), GlialCAM and MLC1 proteins (the latter two are altered in MLC) associate to form a ternary complex when astrocytes are depolarized, such as in situations of high neuronal activity. This association changes the voltage activation of the ClC-2 channel, which leads it to be open to positive potentials, allowing it to participate in the compensation of positive charges in the glial cells introducing chloride.<\/p>\n<p>Thus this work assigns a physiological function to the ClC-2 channel and allows to describe an altered pathophysiological process in MLC, which is necessary to design future therapeutic interventions. This work also hypothesizes what the biochemical function of GlialCAM \/ MLC1 proteins might be.<\/p>\n<p>S\u00f2nia Sirisi, Xabier Elorza-Vidal, Tanit Arnedo, Mercedes Armand-Ug\u00f3n, Gerard Callejo, Xavier Capdevila-Nortes, Tania L\u00f3pez-Hern\u00e1ndez, Uwe Schulte, Alejandro Barrallo-Gimeno, Virginia Nunes, Xavier Gasull, Ra\u00fal Est\u00e9vez. <strong>Depolarization causes the formation of a ternary complex between GlialCAM, MLC1 and ClC-2 in astrocytes: implications in megalencephalic leukoencephalopathy. <\/strong>Human Molecular Genetics doi: 10.1093\/hmg\/ddx134<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Depolarization causes the formation of a ternary complex between GlialCAM, MLC1 and ClC-2 in astrocytes: implications in megalencephalic leukoencephalopathy.<\/p>\n","protected":false},"author":6,"featured_media":7674,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"site-sidebar-layout":"no-sidebar","site-content-layout":"page-builder","ast-site-content-layout":"","site-content-style":"default","site-sidebar-style":"default","ast-global-header-display":"","ast-banner-title-visibility":"","ast-main-header-display":"","ast-hfb-above-header-display":"","ast-hfb-below-header-display":"","ast-hfb-mobile-header-display":"","site-post-title":"","ast-breadcrumbs-content":"","ast-featured-img":"","footer-sml-layout":"","theme-transparent-header-meta":"default","adv-header-id-meta":"","stick-header-meta":"default","header-above-stick-meta":"","header-main-stick-meta":"","header-below-stick-meta":"","astra-migrate-meta-layouts":"default","ast-page-background-enabled":"default","ast-page-background-meta":{"desktop":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center 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