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Authordc.contributor.authorKlein, Andres 
Authordc.contributor.authorMaldonado, Carola 
Authordc.contributor.authorVargas, Lina M. 
Authordc.contributor.authorGonzalez, Marcela 
Authordc.contributor.authorRobledo, Fermín 
Authordc.contributor.authorPerez de Arce, Karen 
Authordc.contributor.authorMuñoz, Francisco J. 
Authordc.contributor.authorHetz Flores, Claudio 
Authordc.contributor.authorAlvarez, Alejandra R. 
Authordc.contributor.authorZanlungo, Silvana 
Cita de ítemdc.identifier.citationNeurobiology of Disease, Volumen 41, Issue 1, 2018, Pages 209-218
Abstractdc.description.abstractNiemann-Pick type C (NPC) is a neurodegenerative disease characterized by the intralysosomal accumulation of cholesterol leading to neuronal apoptosis. We have previously reported the activation of the c-Abl/p73 proapoptotic pathway in the cerebellum of NPC mice; however, upstream signals underlying the engagement of this pathway remain unknown. Here, we investigate the possible role of oxidative stress in the activation of c-Abl/p73 using different in vitro and in vivo NPC models. Our results indicate a close temporal correlation between the appearance of nitrotyrosine (N-Tyr; a post-translational tyrosine modification caused by oxidative stress) and the activation of c-Abl/p73 in NPC models. To test the functional role of oxidative stress in NPC, we have treated NPC neurons with the antioxidant NAC and observed a dramatic decrease of c-Abl/p73 activation and a reduction in the levels of apoptosis in NPC models. In conclusion, our data suggest that oxidative stress is the main upstrea
Type of licensedc.rightsAttribution-NonCommercial-NoDerivs 3.0 Chile
Link to Licensedc.rights.uri
Sourcedc.sourceNeurobiology of Disease
Keywordsdc.subjectNiemann-Pick type C
Keywordsdc.subjectOxidative stress
Títulodc.titleOxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons
Document typedc.typeArtículo de revista
Indexationuchile.indexArtículo de publicación SCOPUS

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Except where otherwise noted, this item's license is described as Attribution-NonCommercial-NoDerivs 3.0 Chile