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dc.creatorMacías, Davides
dc.creatorFernández‐Agüera Rodríguez, Mari Carmenes
dc.creatorBonilla Henao, Victoriaes
dc.creatorLópez Barneo, Josées
dc.date.accessioned2017-09-14T09:57:51Z
dc.date.available2017-09-14T09:57:51Z
dc.date.issued2014-01
dc.identifier.issn17574676es
dc.identifier.urihttp://hdl.handle.net/11441/64426
dc.description.abstractMutations of the von Hippel–Lindau (VHL) gene are associated with pheochromocytomas and paragangliomas, but the role of VHL in sympathoadrenal homeostasis is unknown. We generated mice lacking Vhl in catecholaminergic cells. They exhibited atrophy of the carotid body (CB), adrenal medulla, and sympathetic ganglia. Vhl‐null animals had an increased number of adult CB stem cells, although the survival of newly generated neuron‐like glomus cells was severely compromised. The effects of Vhl deficiency were neither prevented by pharmacological inhibition of prolyl hydroxylases or selective genetic down‐regulation of prolyl hydroxylase‐3, nor phenocopied by hypoxia inducible factor overexpression. Vhl‐deficient animals appeared normal in normoxia but survived for only a few days in hypoxia, presenting with pronounced erythrocytosis, pulmonary edema, and right cardiac hypertrophy. Therefore, in the normal sympathoadrenal setting, Vhl deletion does not give rise to tumors but impairs development and plasticity of the peripheral O2‐sensing system required for survival in hypoxic conditions.es
dc.formatapplication/pdfes
dc.language.isoenges
dc.publisherWileyes
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectAdult carotid body neurogenesises
dc.subjectIntolerance to hypoxiaes
dc.subjectSympathoadrenal tumorigenesises
dc.subjectVhl-deficient mouse modeles
dc.subjectVon Hippel– Lindau proteines
dc.titleDeletion of the von Hippel-Lindau gene causes sympathoadrenal cell death and impairs chemoreceptor-mediated adaptation to hypoxiaes
dc.typeinfo:eu-repo/semantics/articlees
dc.type.versioninfo:eu-repo/semantics/publishedVersiones
dc.rights.accessrightsinfo:eu-repo/semantics/openAccesses
dc.contributor.affiliationInstituto de Biomedicina de Sevilla (IBIS)es
dc.contributor.affiliationUniversidad de Sevilla. Departamento de Fisiología Médica y Biofísicaes
dc.identifier.doi10.15252/emmm.201404153es
idus.format.extent15es
dc.journaltitleEMBO Molecular Medicinees
dc.publication.volumen6es
dc.publication.issue12es
dc.publication.initialPage1577es
dc.publication.endPage1592es

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Except where otherwise noted, this item's license is described as: Attribution-NonCommercial-NoDerivatives 4.0 Internacional