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The Mitochondrial SDHD Gene Is Required for Early Embryogenesis, and Its Partial Deficiency Results in Persistent Carotid Body Glomus Cell Activation with Full Responsiveness to Hypoxia

Opened Access The Mitochondrial SDHD Gene Is Required for Early Embryogenesis, and Its Partial Deficiency Results in Persistent Carotid Body Glomus Cell Activation with Full Responsiveness to Hypoxia
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Autor: Piruat Palomo, José Ignacio
Óscar Pintado, Carmelo
Ortega Sáenz, Patricia
Roche Molina, Marta
López Barneo, José
Departamento: Universidad de Sevilla. Departamento de Fisiología Médica y Biofísica
Fecha: 2004
Publicado en: Molecular and Cellular Biology, Dec. 2004, p. 10933–10940
Tipo de documento: Artículo
Resumen: The SDHD gene encodes one of the two membrane-anchoring proteins of the succinate dehydrogenase (complex II) of the mitochondrial electron transport chain. This gene has recently been proposed to be involved in oxygen sensing because mutations that cause loss of its function produce hereditary familiar paraganglioma, a tumor of the carotid body (CB), the main arterial chemoreceptor that senses oxygen levels in the blood. Here, we report the generation of a SDHD knockout mouse, which to our knowledge is the first mammalian model lacking a protein of the electron transport chain. Homozygous SDHD_/_ animals die at early embryonic stages. Heterozygous SDHD_/_ mice show a general, noncompensated deficiency of succinate dehydrogenase activity without alterations in body weight or major physiological dysfunction. The responsiveness to hypoxia of CBs from SDHD_/_ mice remains intact, although the loss of an SDHD allele results in abnormal enhancement of resting CB activity due to a decrease o...
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URI: http://hdl.handle.net/11441/17484

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