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Deletion of the von Hippel-Lindau gene causes sympathoadrenal cell death and impairs chemoreceptor-mediated adaptation to hypoxia

Opened Access Deletion of the von Hippel-Lindau gene causes sympathoadrenal cell death and impairs chemoreceptor-mediated adaptation to hypoxia

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Autor: Macías, David
Fernández‐Agüera, Mary Carmen
Bonilla Henao, Victoria
López Barneo, José
Departamento: Instituto de Biomedicina de Sevilla (IBIS)
Universidad de Sevilla. Departamento de Fisiología Médica y Biofísica
Fecha: 2014-01
Tipo de documento: Artículo
Resumen: Mutations 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 developm...
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Tamaño: 4.601Mb
Formato: PDF

URI: http://hdl.handle.net/11441/64426

DOI: 10.15252/emmm.201404153

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