Please use this identifier to cite or link to this item: http://hdl.handle.net/2445/133843
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dc.contributor.authorSánchez-Danés, Adriana-
dc.contributor.authorRichaud-Patin, Yvonne-
dc.contributor.authorCarballo Carbajal, Iria-
dc.contributor.authorJiménez-Delgado, Senda-
dc.contributor.authorCaig, Carles-
dc.contributor.authorMora, Sergio-
dc.contributor.authorDi Guglielmo, Claudia-
dc.contributor.authorEzquerra, Mario-
dc.contributor.authorPatel, Bindiben-
dc.contributor.authorGiralt Torroella, Albert-
dc.contributor.authorCanals i Coll, Josep M.-
dc.contributor.authorMemo, Maurizio-
dc.contributor.authorAlberch i Vié, Jordi-
dc.contributor.authorLópez-Barneo, José-
dc.contributor.authorVila Farré, Miquel-
dc.contributor.authorCuervo, Ana Maria-
dc.contributor.authorTolosa, Eduardo-
dc.contributor.authorConsiglio, Antonella-
dc.contributor.authorRaya Chamorro, Ángel-
dc.date.accessioned2019-05-24T16:19:00Z-
dc.date.available2019-05-24T16:19:00Z-
dc.date.issued2012-05-
dc.identifier.issn1757-4676-
dc.identifier.urihttp://hdl.handle.net/2445/133843-
dc.description.abstractInduced pluripotent stem cells (iPSC) offer an unprecedented opportunity to model human disease in relevant cell types, but it is unclear whether they could successfully model age-related diseases such as Parkinson's disease (PD). Here, we generated iPSC lines from seven patients with idiopathic PD (ID-PD), four patients with familial PD associated to the G2019S mutation in the Leucine-Rich Repeat Kinase 2 (LRRK2) gene (LRRK2-PD) and four age- and sex-matched healthy individuals (Ctrl). Over long-time culture, dopaminergic neurons (DAn) differentiated from either ID-PD- or LRRK2-PD-iPSC showed morphological alterations, including reduced numbers of neurites and neurite arborization, as well as accumulation of autophagic vacuoles, which were not evident in DAn differentiated from Ctrl-iPSC. Further induction of autophagy and/or inhibition of lysosomal proteolysis greatly exacerbated the DAn morphological alterations, indicating autophagic compromise in DAn from ID-PD- and LRRK2-PD-iPSC, which we demonstrate occurs at the level of autophagosome clearance. Our study provides an iPSC-based in vitro model that captures the patients' genetic complexity and allows investigation of the pathogenesis of both sporadic and familial PD cases in a disease-relevant cell type.-
dc.format.extent16 p.-
dc.format.mimetypeapplication/pdf-
dc.language.isoeng-
dc.publisherEMBO Press-
dc.relation.isformatofReproducció del document publicat a: https://doi.org/10.1002/emmm.201200215-
dc.relation.ispartofEMBO Molecular Medicine, 2012, vol. 4, num. 5, p. 380-395-
dc.relation.urihttps://doi.org/10.1002/emmm.201200215-
dc.rights(c) EMBO, 2012-
dc.subject.classificationDopamina-
dc.subject.classificationMetabolisme-
dc.subject.classificationFenotip-
dc.subject.classificationGenètica-
dc.subject.classificationNeurones-
dc.subject.classificationMalaltia de Parkinson-
dc.subject.classificationPatologia-
dc.subject.classificationCèl·lules mare-
dc.subject.otherDopamine-
dc.subject.otherMetabolism-
dc.subject.otherPhenotype-
dc.subject.otherGenetics-
dc.subject.otherNeurons-
dc.subject.otherParkinson's disease-
dc.subject.otherPathology-
dc.subject.otherStem cells-
dc.titleDisease-specific phenotypes in dopamine neurons from human iPS-based models of genetic and sporadic Parkinson's disease-
dc.typeinfo:eu-repo/semantics/article-
dc.typeinfo:eu-repo/semantics/publishedVersion-
dc.identifier.idgrec615356-
dc.date.updated2019-05-24T16:19:00Z-
dc.rights.accessRightsinfo:eu-repo/semantics/openAccess-
dc.identifier.pmid22407749-
Appears in Collections:Articles publicats en revistes (Biomedicina)
Articles publicats en revistes (Patologia i Terapèutica Experimental)
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

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