{"id":7800,"date":"2017-04-21T00:00:00","date_gmt":"2017-04-20T22:00:00","guid":{"rendered":"https:\/\/idibell.cat\/blog\/2017\/04\/21\/descriuen-un-nou-proces-fisiopatologic-de-la-leucoencefalopatia-megalencefalica-mlc-en-astrocits\/"},"modified":"2020-05-13T19:46:50","modified_gmt":"2020-05-13T17:46:50","slug":"descriuen-un-nou-proces-fisiopatologic-de-la-leucoencefalopatia-megalencefalica-mlc-en-astrocits","status":"publish","type":"post","link":"https:\/\/idibell.cat\/2017\/04\/descriuen-un-nou-proces-fisiopatologic-de-la-leucoencefalopatia-megalencefalica-mlc-en-astrocits\/","title":{"rendered":"Descriuen un nou proc\u00e9s fisiopatol\u00f2gic de la leucoencefalopatia megalencef\u00e0lica (MLC) en astr\u00f2cits"},"content":{"rendered":"<p>En aquest treball, publicat a la revista <em>Human Molecular Genetics<\/em>, s&#8217;investiga la fisiopatologia de la leucoencefalopatia megalencef\u00e1lica (MLC) en astr\u00f2cits. Els primers autors del treball s\u00f3n Sonia Sirisi, Xabier Elorza-Vidal i Tanit Arnedo, adscrits a UB-IDIBELL i CIBERER. El treball ha estat codirigit per Xavier Gasull (Institut de Neuroci\u00e8ncies-UB i IDIBAPS) i Ra\u00fal Est\u00e9vez (Institut de Neuroci\u00e8ncies-UB, l&#8217;IDIBELL i CIBERER), en col\u00b7laboraci\u00f3 amb Virginia Nunes (UB, l&#8217;IDIBELL i CIBERER).<\/p>\n<p>L\u2019estudi ha perm\u00e8s concloure que la prote\u00efna ClC-2 (canal de clorur), GlialCAM i MLC1 (les dues darreres estan alterades en MLC) s&#8217;associen formant un complex ternari quan els astr\u00f2cits estan despolaritzats, com passa en situacions d&#8217;alta activitat neuronal. Aquesta associaci\u00f3 canvia l&#8217;activaci\u00f3 per voltatge del canal ClC-2, fet que el que el porta a estar obert a potencials positius i participar en la compensaci\u00f3 de c\u00e0rregues positives en les c\u00e8l\u00b7lules glials introduint clorur. Aix\u00ed, aquest treball assigna una funci\u00f3 fisiol\u00f2gica al canal ClC-2 i permet descriure un proc\u00e9s fisiopatol\u00f2gic alterat en MLC, qualcom necessari per a dissenyar futures intervencions terap\u00e8utiques. En aquest treball tamb\u00e9 s&#8217;hipotetitza sobre la funci\u00f3 bioqu\u00edmica de les prote\u00efnes GlialCAM \/ MLC1.<\/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\/ddx134uyt<\/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":10666,"comment_status":"closed","ping_status":"closed","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 center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"tablet":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"mobile":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""}},"ast-content-background-meta":{"desktop":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"tablet":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"mobile":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""}},"footnotes":""},"categories":[1,465],"tags":[],"publishpress_future_action":{"enabled":false,"date":"2024-05-09 14:38:25","action":"change-status","newStatus":"draft","terms":[],"taxonomy":"category"},"_links":{"self":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/7800"}],"collection":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/users\/6"}],"replies":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/comments?post=7800"}],"version-history":[{"count":0,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/7800\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/media\/10666"}],"wp:attachment":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/media?parent=7800"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/categories?post=7800"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/tags?post=7800"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}