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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
4
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pubmed:dateCreated |
2007-9-27
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pubmed:abstractText |
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a progressive cerebral white matter disease with onset in childhood, caused by mutations in the MLC1 gene. MLC1 is a protein with unknown function that is mainly expressed in the brain in astrocytic endfeet at the blood-brain and cerebrospinal fluid-brain barriers. It shares its localization at astrocytic endfeet with the dystrophin-associated glycoprotein complex (DGC). The objective of the present study was to investigate the possible association of MLC1 with the DGC. To test this hypothesis, (co)-localization of DGC-proteins and MLC1 was analyzed by immunohistochemical stainings in gliotic brain tissue from a patient with multiple sclerosis, in glioblastoma tissue and in brain tissue from an MLC patient. In control tissue, a direct protein interaction was tested by immunoprecipitation. Results revealed that MLC1 is co-localized with DGC-proteins in gliotic brain tissue. We demonstrated that both MLC1 and aquaporin-4, a member of the DGC, were redistributed in glioblastoma cells. In MLC brain tissue, we showed absence of MLC1 and altered expression of several DGC-proteins. We demonstrated a direct protein interaction between MLC1 and Kir4.1. From these results we conclude that MLC1 is associated with the DGC at astrocytic endfeet.
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-10595940,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-10719269,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-11254442,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-11377836,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-11516613,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-11592034,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-11717465,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-12140558,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-14507673,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-14735305,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-15021250,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-15102837,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-15367490,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-15561407,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-15723236,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-15892299,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-16652334,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-16825957,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-16868787,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-17057071,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-3278318,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-6517223,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-7550355,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-7695231,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-8841668,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-8996823,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-9225685,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17628813-9270600
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/AQP4 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Aquaporin 4,
http://linkedlifedata.com/resource/pubmed/chemical/Dystrophin-Associated Protein...,
http://linkedlifedata.com/resource/pubmed/chemical/Glycoproteins,
http://linkedlifedata.com/resource/pubmed/chemical/MLC1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Membrane Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Potassium Channels, Inwardly...,
http://linkedlifedata.com/resource/pubmed/chemical/potassium inwardly-rectifying...
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pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
0001-6322
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pubmed:author |
pubmed-author:BoorIljaI,
pubmed-author:BoveKevin EKE,
pubmed-author:DinopoulosArgiriosA,
pubmed-author:EstévezRaúlR,
pubmed-author:KamphorstWouterW,
pubmed-author:MuntoniFrancescoF,
pubmed-author:NagtegaalMachielM,
pubmed-author:Pascual-CastroviejoIgnacioI,
pubmed-author:PronkJan CJC,
pubmed-author:ScheperGert CGC,
pubmed-author:van HorssenJackJ,
pubmed-author:van der KnaapMarjo SMS,
pubmed-author:van der ValkPaulP
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pubmed:issnType |
Print
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pubmed:volume |
114
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
403-10
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pubmed:dateRevised |
2010-9-16
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pubmed:meshHeading |
pubmed-meshheading:17628813-Aquaporin 4,
pubmed-meshheading:17628813-Astrocytes,
pubmed-meshheading:17628813-Blotting, Western,
pubmed-meshheading:17628813-Brain,
pubmed-meshheading:17628813-Brain Neoplasms,
pubmed-meshheading:17628813-Central Nervous System Cysts,
pubmed-meshheading:17628813-Dystrophin-Associated Protein Complex,
pubmed-meshheading:17628813-Glioma,
pubmed-meshheading:17628813-Glycoproteins,
pubmed-meshheading:17628813-Heredodegenerative Disorders, Nervous System,
pubmed-meshheading:17628813-Humans,
pubmed-meshheading:17628813-Immunohistochemistry,
pubmed-meshheading:17628813-Immunoprecipitation,
pubmed-meshheading:17628813-Membrane Proteins,
pubmed-meshheading:17628813-Multiple Sclerosis,
pubmed-meshheading:17628813-Potassium Channels, Inwardly Rectifying
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pubmed:year |
2007
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pubmed:articleTitle |
MLC1 is associated with the dystrophin-glycoprotein complex at astrocytic endfeet.
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pubmed:affiliation |
Department of Pediatrics/Child Neurology, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands. ms.vanderknaap@vumc.nl
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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