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http://purl.uniprot.org/citations/20477942http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/20477942http://www.w3.org/2000/01/rdf-schema#comment"A dominant mutation in the gene coding for the vesicle-associated membrane protein-associated protein B (VAPB) was associated with amyotrophic lateral sclerosis, a fatal paralytic disorder characterized by the selective loss of motoneurons in the brain and spinal cord. Adeno-associated viral vectors that we show to transduce up to 90% of motoneurons in vitro were used to model VAPB-associated neurodegenerative process. We observed that Adeno-associated viral-mediated over-expression of both wild-type and mutated form of human VAPB selectively induces death of primary motoneurons, albeit with different kinetics. We provide evidence that ER stress and impaired homeostatic regulation of calcium (Ca(2+)) are implicated in the death process. Finally, we found that completion of the motoneuron death program triggered by the over-expression of wild-type and mutant VAPB implicates calpains, caspase 12 and 3. Our viral-based in vitro model, which recapitulates the selective vulnerability of motoneurons to the presence of mutant VAPB and also to VAPB gene dosage effect, identifies aberrant Ca(2+) signals and ER-derived death pathways as important events in the motoneuron degenerative process."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.org/dc/terms/identifier"doi:10.1111/j.1471-4159.2010.06806.x"xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/author"Moumen A."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/author"Medina I."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/author"Raoul C."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/author"Pellegrino C."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/author"Aebischer P."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/author"Aebischer J."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/author"Langou K."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/date"2010"xsd:gYear
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/name"J Neurochem"xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/pages"795-809"xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/title"AAV-mediated expression of wild-type and ALS-linked mutant VAPB selectively triggers death of motoneurons through a Ca2+-dependent ER-associated pathway."xsd:string
http://purl.uniprot.org/citations/20477942http://purl.uniprot.org/core/volume"114"xsd:string
http://purl.uniprot.org/citations/20477942http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/20477942
http://purl.uniprot.org/citations/20477942http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/20477942
http://purl.uniprot.org/uniprot/#_Q3TU78-mappedCitation-20477942http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/20477942
http://purl.uniprot.org/uniprot/#_Q8BR39-mappedCitation-20477942http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/20477942
http://purl.uniprot.org/uniprot/#_Q8BH80-mappedCitation-20477942http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/20477942
http://purl.uniprot.org/uniprot/#_Q3TA54-mappedCitation-20477942http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/20477942
http://purl.uniprot.org/uniprot/#_Q9QY76-mappedCitation-20477942http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/20477942
http://purl.uniprot.org/uniprot/Q3TA54http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/20477942
http://purl.uniprot.org/uniprot/Q9QY76http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/20477942
http://purl.uniprot.org/uniprot/Q8BH80http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/20477942