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http://hdl.handle.net/2445/129370
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DC Field | Value | Language |
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dc.contributor.author | Hoegg-Beiler, Maja B. | - |
dc.contributor.author | Sirisi Dolcet, Sònia | - |
dc.contributor.author | Orozco, Ian J. | - |
dc.contributor.author | Ferrer, Isidro (Ferrer Abizanda) | - |
dc.contributor.author | Hohensee, Svea | - |
dc.contributor.author | Auberson, Muriel | - |
dc.contributor.author | Gödde, Kathrin | - |
dc.contributor.author | Vilches, Clara | - |
dc.contributor.author | López de Heredia, Miguel | - |
dc.contributor.author | Nunes Martínez, Virginia | - |
dc.contributor.author | Estévez Povedano, Raúl | - |
dc.contributor.author | Jentsch, Thomas J. | - |
dc.date.accessioned | 2019-02-28T12:51:05Z | - |
dc.date.available | 2019-02-28T12:51:05Z | - |
dc.date.issued | 2014 | - |
dc.identifier.issn | 2041-1723 | - |
dc.identifier.uri | http://hdl.handle.net/2445/129370 | - |
dc.description.abstract | Defects in the astrocytic membrane protein MLC1, the adhesion molecule GlialCAM or the chloride channel ClC-2 underlie human leukoencephalopathies. Whereas GlialCAM binds ClC-2 and MLC1, and modifies ClC-2 currents in vitro, no functional connections between MLC1 and ClC-2 are known. Here we investigate this by generating loss-of-function Glialcam and Mlc1 mouse models manifesting myelin vacuolization. We find that ClC-2 is unnecessary for MLC1 and GlialCAM localization in brain, whereas GlialCAM is important for targeting MLC1 and ClC-2 to specialized glial domains in vivo and for modifying ClC-2's biophysical properties specifically in oligodendrocytes (OLs), the cells chiefly affected by vacuolization. Unexpectedly, MLC1 is crucial for proper localization of GlialCAM and ClC-2, and for changing ClC-2 currents. Our data unmask an unforeseen functional relationship between MLC1 and ClC-2 in vivo, which is probably mediated by GlialCAM, and suggest that ClC-2 participates in the pathogenesis of megalencephalic leukoencephalopathy with subcortical cysts. | - |
dc.format.extent | 17 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Nature Publishing Group | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1038/ncomms4475 | - |
dc.relation.ispartof | Nature Communications, 2014, vol. 5, p. 3475 | - |
dc.relation.uri | https://doi.org/10.1038/ncomms4475 | - |
dc.rights | cc-by (c) Hoegg-Beiler, Maja B. et al., 2014 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es | - |
dc.source | Articles publicats en revistes (Ciències Fisiològiques) | - |
dc.subject.classification | Malalties cerebrals | - |
dc.subject.classification | Teixit nerviós | - |
dc.subject.classification | Metabolisme cel·lular | - |
dc.subject.classification | Proteïnes de membrana | - |
dc.subject.classification | Canals de clorur | - |
dc.subject.other | Brain diseases | - |
dc.subject.other | Nerve tissue | - |
dc.subject.other | Cell metabolism | - |
dc.subject.other | Membrane proteins | - |
dc.subject.other | Chloride channels | - |
dc.title | Disrupting MLC1 and GlialCAM and ClC-2interactions in leukodystrophy entails glial chloridechannel dysfunction | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 636266 | - |
dc.date.updated | 2019-02-28T12:51:05Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 24647135 | - |
Appears in Collections: | Articles publicats en revistes (Patologia i Terapèutica Experimental) Articles publicats en revistes (Ciències Fisiològiques) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
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636266.pdf | 4.12 MB | Adobe PDF | View/Open |
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