Lack of Activation of the Unfolded Protein Response in Mouse and Cellular Models of Niemann-Pick Type C Disease

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dc.catalogadorjlo
dc.contributor.authorKlein Posternack, Andrés David
dc.contributor.authorMosqueira Montero, Matías José
dc.contributor.authorMartínez, Gabriela
dc.contributor.authorRobledo Plaza, Fermín Alberto
dc.contributor.authorGonzález Bustos, Marcela Paz
dc.contributor.authorCaballero, Benjamín
dc.contributor.authorCancino Lobos, Gonzalo
dc.contributor.authorÁlvarez Rojas, Alejandra Beatriz
dc.contributor.authorHetz, Claudio
dc.contributor.authorZanlungo Matsuhiro, Silvana
dc.date.accessioned2024-04-09T20:51:59Z
dc.date.available2024-04-09T20:51:59Z
dc.date.issued2011
dc.description.abstractBackground: Niemann-Pick type C (NPC) disease is a fatal lysosomal storage disease related to progressive neurode-generation secondary to abnormal intracellular accumulation of cholesterol. Signs of endoplasmic reticulum (ER) stress have been reported in other lipidoses. Adaptation to ER stress is mediated by the unfolded protein response (UPR), an integrated signal transduction pathway that attenuates stress or triggers apoptosis of irreversibly damaged cells. Objective: To investigate the possible engagement of ER stress responses in NPC models. Methods: We used NPC1 deficient mice and an NPC cell-based model by knocking down the expression of NPC1 to measure several UPR markers through different approaches. Results: Despite expectations that the UPR will be activated in NPC, our results indicate a lack of ER stress reactions in the cerebellum of symptomatic mice. Similarly, knocking down NPC1 in Neuro2a cells leads to clear cholesterol accumulation without evidence of UPR activation. Conclusion: Our results suggest that cholesterol overload and neuronal dysfunction in NPC is not associated with ER stress, which contrasts with recent reports suggesting the activation of the UPR in other lysosomal storage diseases. Copyright (c) 2010 S. Karger AG, Basel
dc.format.extent5 páginas
dc.fuente.origenConveris
dc.identifier.doi10.1159/000316540
dc.identifier.eissn1660-2862
dc.identifier.issn1660-2854
dc.identifier.pubmedid20714112
dc.identifier.urihttp://dx.doi.org/10.1159/000316540
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/85014
dc.identifier.wosidWOS:000289100800004
dc.information.autorucFacultad de Ciencias Biológicas; Klein Posternack, Andrés David; 0000-0002-9951-2174; 2966
dc.information.autorucFacultad de Ciencias Biológicas; Mosqueira Montero, Matías José; 0000-0001-7744-9138; 1231
dc.information.autorucFacultad de Ciencias Biológicas; Robledo Plaza, Fermín Alberto; S/I; 131967
dc.information.autorucFacultad de Ciencias Biológicas; González Bustos, Marcela Paz; S/I; 1011022
dc.information.autorucFacultad de Ciencias Biológicas; Cancino Lobos, Gonzalo; 0000-0001-8697-7282; 17709
dc.information.autorucFacultad de Ciencias Biológicas; Álvarez Rojas, Alejandra Beatriz; 0000-0002-8129-9280; 83681
dc.information.autorucFacultad de Ciencias Biológicas; Zanlungo Matsuhiro, Silvana; 0000-0001-8383-9829; 72650
dc.issue.numero3
dc.language.isoen
dc.nota.accesocontenido parcial
dc.pagina.final128
dc.pagina.inicio124
dc.revistaNeurodegenerative diseases
dc.rightsacceso restringido
dc.subjectApoptosis
dc.subjectCholesterol
dc.subjectEndoplasmic reticulum stress
dc.subject.ddc610
dc.subject.deweyMedicina y saludes_ES
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleLack of Activation of the Unfolded Protein Response in Mouse and Cellular Models of Niemann-Pick Type C Disease
dc.typeartículo
dc.volumen8
sipa.codpersvinculados127295
sipa.codpersvinculados142326
sipa.codpersvinculados2966
sipa.codpersvinculados1231
sipa.codpersvinculados131967
sipa.codpersvinculados1011022
sipa.codpersvinculados17709
sipa.codpersvinculados83681
sipa.codpersvinculados72650
sipa.trazabilidadConveris;20-07-2021
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