{"id":10447,"date":"2017-04-21T00:00:00","date_gmt":"2017-04-20T22:00:00","guid":{"rendered":"https:\/\/idibell.cat\/es\/blog\/2017\/04\/21\/describen-un-nuevo-proceso-fisiopatologico-de-la-leucoencefalopatia-megalencefalica-mlc-en-astrocitos\/"},"modified":"2020-05-20T08:03:48","modified_gmt":"2020-05-20T06:03:48","slug":"describen-un-nuevo-proceso-fisiopatologico-de-la-leucoencefalopatia-megalencefalica-mlc-en-astrocitos","status":"publish","type":"post","link":"https:\/\/idibell.cat\/es\/2017\/04\/describen-un-nuevo-proceso-fisiopatologico-de-la-leucoencefalopatia-megalencefalica-mlc-en-astrocitos\/","title":{"rendered":"Describen un nuevo proceso fisiopatol\u00f3gico de la leucoencefalopat\u00eda megalencef\u00e1lica (MLC) en astrocitos"},"content":{"rendered":"<p>En este trabajo, publicado en la revista Human Molecular Genetics, donde los primeros autores del trabajo son Sonia Sirisi, Xabier Elorza-Vidal y Tanit Arnedo -pertenencientes a UB-IDIBELL y CIBERER-, se investiga la fisiopatolog\u00eda de la leucoencefalopat\u00eda megalencef\u00e1lica (MLC) en astrocitos. El trabajo ha sido codirigido por Xavier Gasull (Instituto de Neurociencias-UB y IDIBAPS) y Ra\u00fal Est\u00e9vez (Instituto de Neurociencias-UB, IDIBELL y CIBERER), en colaboraci\u00f3n con Virginia Nunes (UB, IDIBELL y CIBERER). <b><br \/> El trabajo ha permitido concluir que la prote\u00edna ClC-2 (canal de cloruro), GlialCAM y MLC1 (estas dos \u00faltimas estan alteradas en MLC) se asocian formando un complejo ternario cuando los astrocitos est\u00e1n despolarizados, como ocurre en situaciones de alta actividad neuronal. Esta asociaci\u00f3n cambia la activaci\u00f3n por voltaje del canal ClC-2, lo que le lleva a estar abierto a potenciales positivos, permiti\u00e9ndole participar en la compensaci\u00f3n de cargas positivas en las c\u00e9lulas gliales introduciendo cloruro.<b><br \/> <b><br \/> As\u00ed, este trabajo asigna una funci\u00f3n fisiol\u00f3gica al canal ClC-2 y permite describir un proceso fisiopatol\u00f3gico alterado en MLC, lo cu\u00e1l es necesario para dise\u00f1ar futuras intervenciones terap\u00e9uticas. En este trabajo tambi\u00e9n se hipotetiza cual podr\u00eda ser la funci\u00f3n bioqu\u00edmica de las prote\u00ednas GlialCAM\/MLC1. <b><br \/> S\u00f2nia Sirisi, Xabier Elorza-Vidal, Tanit Arnedo, Mercedes Armand-Ug\u00f3n, Gerard Callejo, Xavier Capdevila-Nortes, Tania L\u00f3pez-Hern\u00e1ndez, Uwe Schulte,<sup> <\/sup>Alejandro Barrallo-Gimeno, Virginia Nunes, Xavier Gasull, Ra\u00fal Est\u00e9vez.<strong> <\/strong><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":10448,"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 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