ArticleEMBO molecular medicine2014
Deletion of the von Hippel-Lindau gene causes sympathoadrenal cell death and impairs chemoreceptor-mediated adaptation to hypoxia.
Article in EMBO molecular medicine, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 54 citations in OpenAlex.
- Exacerbated ATP transmission in the carotid body is linked to glomus cell expansion in spontaneously hypertensive rats.The Journal of physiology · 2026Article
- Genetic map of the carotid body stem cell niche with focus on the OScientific reports · 2025Article
- Hif-2α programs oxygen chemosensitivity in chromaffin cells.The Journal of clinical investigation · 2024Article
- Extracellular matrix protein anosmin-1 overexpression alters dopaminergic phenotype in the CNS and the PNS with no pathogenic consequences in a MPTP model of Parkinson's disease.Brain structure & function · 2023Article
- Stem Cell Niche in the Mammalian Carotid Body.Advances in anatomy, embryology, and cell biology · 2023Article
- HIF and MYC signaling in adrenal neoplasms of the neural crest: implications for pediatrics.Frontiers in endocrinology · 2023Review
- Constitutive Expression of Hif2α Confers Acute OAdvances in experimental medicine and biology · 2023Article
- HIF2α regulates the synthesis and release of epinephrine in the adrenal medulla.Journal of molecular medicine (Berlin, Germany) · 2021Article
- Developmental role of PHD2 in the pathogenesis of pseudohypoxic pheochromocytoma.Endocrine-related cancer · 2021Article
- Comparative morphological and molecular studies on the oxygen-chemoreceptive cells in the carotid body and fish gills.Cell and tissue research · 2021Review
- Targeting HIF2α-ARNT hetero-dimerisation as a novel therapeutic strategy for pulmonary arterial hypertension.The European respiratory journal · 2021Article
- Marked and rapid effects of pharmacological HIF-2α antagonism on hypoxic ventilatory control.The Journal of clinical investigation · 2020Article
- Molecular Mechanisms of Acute Oxygen Sensing by Arterial Chemoreceptor Cells. Role of Hif2α.Frontiers in physiology · 2020Review
- EglN3 hydroxylase stabilizes BIM-EL linking VHL type 2C mutations to pheochromocytoma pathogenesis and chemotherapy resistance.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Mitochondrial Complex I Function Is Essential for Neural Stem/Progenitor Cells Proliferation and Differentiation.Frontiers in neuroscience · 2019Article
- The role of Olfr78 in the breathing circuit of mice.Nature · 2018Article
- PHD2 inactivation in Type I cells drives HIF-2α-dependent multilineage hyperplasia and the formation of paraganglioma-like carotid bodies.The Journal of physiology · 2018Article
- Rodent models of pheochromocytoma, parallels in rodent and human tumorigenesis.Cell and tissue research · 2018Review
- Metabolic implications of hypoxia and pseudohypoxia in pheochromocytoma and paraganglioma.Cell and tissue research · 2018Review
- Oxygen sensing and stem cell activation in the hypoxic carotid body.Cell and tissue research · 2018Review
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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 development and plasticity of the peripheral O₂-sensing system required for survival in hypoxic conditions.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.