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http://purl.uniprot.org/citations/22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/22127389http://www.w3.org/2000/01/rdf-schema#comment"Mecp2 deficiency or overexpression causes a wide spectrum of neurological diseases in humans among which Rett Syndrome is the prototype. Pathogenic mechanisms are thought to involve transcriptional deregulation of target genes such as Bdnf together with defects in the general transcriptional program of affected cells. Here we found that two master genes, Huntingtin (Htt) and huntingtin-associated protein (Hap1), involved in the control of Bdnf axonal transport, are altered in the brain of Mecp2-deficient mice. We also revealed an in vivo defect of Bdnf transport throughout the cortico striatal pathway of Mecp2-deficient animals. We found that the velocity of Bdnf-containing vesicles is reduced in vitro in the Mecp2-deficient axons and this deficit can be rescued by the re-expression of Mecp2. The defect in axonal transport is not restricted to Bdnf since transport of the amyloid precursor protein (App) that is Htt and Hap1-dependent is also altered. Finally, treating Mecp2-deficient mice with cysteamine, a molecule increasing the secretion of Bdnf vesicles, improved the lifespan and reduced motor defects, suggesting a new therapeutic strategy for Rett syndrome."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.org/dc/terms/identifier"doi:10.1016/j.nbd.2011.11.002"xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/author"Saudou F."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/author"Villard L."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/author"Borges-Correia A."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/author"Roux J.C."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/author"Zala D."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/author"Panayotis N."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/date"2012"xsd:gYear
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/name"Neurobiol Dis"xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/pages"786-795"xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/title"Modification of Mecp2 dosage alters axonal transport through the Huntingtin/Hap1 pathway."xsd:string
http://purl.uniprot.org/citations/22127389http://purl.uniprot.org/core/volume"45"xsd:string
http://purl.uniprot.org/citations/22127389http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/22127389
http://purl.uniprot.org/citations/22127389http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/22127389
http://purl.uniprot.org/uniprot/Q00566#attribution-4F3120FD85654F70AFBA47B19CA2BF45http://purl.uniprot.org/core/sourcehttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/#_A0A0G2JWK2-mappedCitation-22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/#_D3Z7U4-mappedCitation-22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/#_D3YY81-mappedCitation-22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/#_A0A8I6ABA1-mappedCitation-22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/#_Q09GN5-mappedCitation-22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/#_Q00566-mappedCitation-22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/#_Q9Z2D6-mappedCitation-22127389http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/22127389
http://purl.uniprot.org/uniprot/D3YY81http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/22127389