RT Journal Article
T1 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.
A1 El-Bekay, Rajaa
A1 Romero-Zerbo, Silvana Yanina
A1 Decara, Juan
A1 Sánchez-Salido, Lourdes
A1 Del Arco-Herrera, Ignacio
A1 Rodriguez-de-Fonseca, Fernando
A1 De Diego‑Otero, Yolanda
K1 Estrés oxidativo - Modelos animales
K1 Enfermedades hereditarias
AB 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.
PB Wiley
YR 2007
FD 2007
LK https://hdl.handle.net/10630/32396
UL https://hdl.handle.net/10630/32396
LA eng
NO 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
NO Política de acceso abierto tomada de: https://v2.sherpa.ac.uk/id/publication/6992
DS RIUMA. Repositorio Institucional de la Universidad de Málaga
RD 20 ene 2026