2026-06-07T05:49:41Zhttps://riuma.uma.es/rest/oai/request

oai:riuma.uma.es:10630/323962026-02-03T11:00:13Zcom_10630_2254col_10630_37953

Enhanced markers of oxidative stress, altered antioxidants and NADPH-oxidase activation in brains from Fragile X mental retardation 1-deficient mice, a pathological model for Fragile X syndrome. El-Bekay, Rajaa Romero-Zerbo, Silvana Yanina Decara, Juan Sánchez-Salido, Lourdes Del Arco-Herrera, Ignacio Rodriguez-de-Fonseca, Fernando De Diego‑Otero, Yolanda Estrés oxidativo - Modelos animales Enfermedades hereditarias Fragile X syndrome is the most common form of inherited mental retardation in humans. It originates from the loss of expression ofthe Fragile X mental retardation 1 (FMR1) gene, which results in the absence of the Fragile X mental retardation protein. However,the biochemical mechanisms involved in the pathological phenotype are mostly unknown. The availability of the FMR1-knockoutmouse model offers an excellent model system in which to study the biochemical alterations related to brain abnormalities in thesyndrome. We show for the first time that brains from Fmr1-knockout mice, a validated model for the syndrome, display higher levelsof reactive oxygen species, nicotinamide adenine dinucleotide phosphate (NADPH)-oxidase activation, lipid peroxidation and proteinoxidation than brains from wild-type mice. Furthermore, the antioxidant system is deficient in Fmr1-knockout mice, as shown byaltered levels of components of the glutathione system. FMR1-knockout mice lacking Fragile X mental retardation protein werecompared with congenic FVB129 wild-type controls. Our results support the hypothesis that the lack of Fragile X mental retardationprotein function leads to a moderate increase of the oxidative stress status in the brain that may contribute to the pathophysiology ofthe Fragile X syndrome. 2024-08-01T10:52:22Z 2024-08-01T10:52:22Z 2024-08-01T10:52:22Z 2007 journal article El Bekay, R., Romero-Zerbo, Y., Decara, J., Sanchez-Salido, L., Del Arco-Herrera, I., Rodríguez-de Fonseca, F. and De Diego-Otero, Y. (2007), Enhanced markers of oxidative stress, altered antioxidants and NADPH-oxidase activation in brains from Fragile X mental retardation 1-deficient mice, a pathological model for Fragile X syndrome. European Journal of Neuroscience, 26: 3169-3180. https://doi.org/10.1111/j.1460-9568.2007.05939.x https://hdl.handle.net/10630/32396 10.1111/j.1460-9568.2007.05939.x eng http://creativecommons.org/licenses/by/4.0/ open access Atribución 4.0 Internacional Wiley