Propranolol reduces viability and induces apoptosis in hemangioblastoma cells from von Hippel-Lindau patients

[Background] Von Hippel-Lindau (VHL) disease is a rare oncological disease with an incidence of 1:36,000, and is characterized by the growth of different types of tumors: hemangioblastomas in the central nervous system (CNS) and retina, renal carcinoma, pheochromocytomas, pancreatic serous cystadeno...

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Detalles Bibliográficos
Autores: Albiñana, Virginia, Villar Gómez de las Heras, Karina, Serrano-Heras, Gemma, Segura, Tomás, Perona-Moratalla, Ana Belén, Mota-Pérez, Mercedes, Campos, José María de, Botella, Luisa María
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2015
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/125813
Acceso en línea:http://hdl.handle.net/10261/125813
Access Level:acceso abierto
Palabra clave:von Hippel-Lindau disease (VHL)
pVHL
Hypoxia inducible factor
hemangioblastoma
CNS tumors
Propranolol
Descripción
Sumario:[Background] Von Hippel-Lindau (VHL) disease is a rare oncological disease with an incidence of 1:36,000, and is characterized by the growth of different types of tumors: hemangioblastomas in the central nervous system (CNS) and retina, renal carcinoma, pheochromocytomas, pancreatic serous cystadenoma, and endolymphatic sac tumors. These tumors do not express VHL protein (pVHL). pVHL ubiquitinates hypoxia inducible factor (HIF) protein for degradation by the proteasome; in the absence of VHL, HIF translocates to the nucleus to activate the expression of its target genes. Targeting VHL-derived tumors with drugs that have reduced side effects is urgent to avoid repeat CNS surgeries. Recent reports have shown that propranolol, a β-blocker used for the treatment of hypertension and other cardiac and neurological diseases, is the best option for infantile hemangioma (IH). Propranolol could be an efficient treatment to control hemangioblastoma growth in VHL disease because of its antiangiogenic effects demonstrated in IH and the hypothetical impact on HIF levels.