RT Journal Article
T1 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.
A1 Lima-Cabello, Elena
A1 Garcia-Guirado, Francisco
A1 Calvo-Medina, Rocio
A1 el Bekay, Rajaa
A1 Perez-Costillas, Lucia
A1 Quintero-Navarro, Carolina
A1 Sanchez-Salido, Lourdes
A1 de Diego-Otero, Yolanda
K1 Fragile X syndrome
K1 Nitric oxide metabolism
K1 Protein nitration
K1 Nitric oxide synthase
K1 Nuclear factor kappa B
K1 Brain
K1 Mouse model
K1 Oxidative stress
AB 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.
PB Hindawi Publishing Corporation
YR 2016
FD 2016
LK http://hdl.handle.net/10668/2401
UL http://hdl.handle.net/10668/2401
LA en
NO Lima-Cabello E, Garcia-Guirado F, Calvo-Medina R, el Bekay R, Perez-Costillas L, Quintero-Navarro C, et al. 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. Oxid Med Cell Longev; 2016:8548910
DS RISalud
RD Aug 30, 2025