2026-04-29T12:25:15Zhttps://repisalud.isciii.es/rest/oai/request
oai:repisalud.isciii.es:20.500.12105/171172024-11-28T15:08:23Zcom_20.500.12105_15322com_20.500.12105_2051col_20.500.12105_16927
Repisalud author Lima-Cabello, Elena author Garcia-Guirado, Francisco author Calvo-Medina, Rocio author el Bekay, Rajaa author Perez-Costillas, Lucia author Quintero-Navarro, Carolina author Sanchez-Salido, Lourdes author de Diego-Otero, Yolanda authoraffiliation [Lima-Cabello,E; Garcia-Guirado,F; Perez-Costillas,L; Quintero-Navarro,C; Sanchez-Salido,L; de Diego-Otero,Y] UGC Salud Mental, Hospital Regional Universitario de Málaga, IBIMA Institute, University of Málaga, Research Laboratory, Hospital Civil, Málaga, Spain. [Lima-Cabello,E] Plant Reproductive Biology Group, Department of Biochemistry, Cell and Molecular Biology of Plants, Estacion Experimental del Zaidin, Spanish Council for Scientific Research (CSIC), Granada, Spain. [Calvo-Medina,R] UGC Pediatría, Sección Neuropediatria, Hospital Regional Universitario Málaga, Hospital Materno-Infantil, Málaga, Spain. [el Bekay,R] UGC Endocrinologia y Nutrición, Instituto de Investigacion Biomedica de Málaga (IBIMA), Hospital Regional Universitario de Málaga, Universidad de Málaga, CIBER de Fisiopatología de la Obesidad y Nutrición (CIBERobn), Instituto de Salud Carlos III (ISCIII), Hospital Civil, Málaga, Spain. [Perez-Costillas,L] Departamento de Salud Publica y Psiquiatría, Universidad de Málaga, Málaga, Spain. 2024-01-16T12:15:52Z 2024-01-16T12:15:52Z 2016 10.1155/2016/8548910 1942-0994 Oxidative Medicine and Cellular Longevity http://hdl.handle.net/10668/2401 26788253 http://hdl.handle.net/20.500.12105/17117 BACKGROUND: Fragile X syndrome is the most common genetic cause of mental disability. Although many research has been performed, the mechanism underlying the pathogenesis is unclear and needs further investigation. Oxidative stress played major roles in the syndrome. The aim was to investigate the nitric oxide metabolism, protein nitration level, the expression of NOS isoforms, and furthermore the activation of the nuclear factor NF-κB-p65 subunit in different brain areas on the fragile X mouse model. METHODS: This study involved adult male Fmr1-knockout and wild-type mice as controls. We detected nitric oxide metabolism and the activation of the nuclear factor NF-κBp65 subunit, comparing the mRNA expression and protein content of the three NOS isoforms in different brain areas. RESULTS: Fmr1-KO mice showed an abnormal nitric oxide metabolism and increased levels of protein tyrosine nitrosylation. Besides that, nuclear factor NF-κB-p65 and inducible nitric oxide synthase appeared significantly increased in the Fmr1-knockout mice. mRNA and protein levels of the neuronal nitric oxide synthase appeared significantly decreased in the knockout mice. However, the epithelial nitric oxide synthase isoform displayed no significant changes. CONCLUSIONS: These data suggest the potential involvement of an abnormal nitric oxide metabolism in the pathogenesis of the fragile X syndrome. eng Animales Encéfalo Modelos de enfermedad en animales Síndrome del cromosoma X frágil Masculino Ratones Ratones noqueados Óxido nítrico Óxido nítrico sintasa Estrés oxidativo Isoformas de proteínas ARN mensajero Factor de transcripción ReIA Tirosina An Abnormal Nitric Oxide Metabolism Contributes to Brain Oxidative Stress in the Mouse Model for the Fragile X Syndrome, a Possible Role in Intellectual Disability. research article