Publication: 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.
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Identifiers
Date
2016
Authors
Lima-Cabello, Elena
Garcia-Guirado, Francisco
Calvo-Medina, Rocio
el Bekay, Rajaa
Perez-Costillas, Lucia
Quintero-Navarro, Carolina
Sanchez-Salido, Lourdes
de Diego-Otero, Yolanda
Advisors
Journal Title
Journal ISSN
Volume Title
Publisher
Hindawi Publishing Corporation
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Abstract
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.
Description
MeSH Terms
Medical Subject Headings::Organisms::Eukaryota::Animals
Medical Subject Headings::Anatomy::Nervous System::Central Nervous System::Brain
Medical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Models, Animal::Disease Models, Animal
Medical Subject Headings::Diseases::Congenital, Hereditary, and Neonatal Diseases and Abnormalities::Genetic Diseases, Inborn::Genetic Diseases, X-Linked::Mental Retardation, X-Linked::Fragile X Syndrome
Medical Subject Headings::Check Tags::Male
Medical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Rodentia::Muridae::Murinae::Mice
Medical Subject Headings::Organisms::Eukaryota::Animals::Animal Population Groups::Animals, Genetically Modified::Mice, Transgenic::Mice, Knockout
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Transcription Factors::NF-kappa B
Medical Subject Headings::Chemicals and Drugs::Inorganic Chemicals::Nitrogen Compounds::Nitrogen Oxides::Nitric Oxide
Medical Subject Headings::Chemicals and Drugs::Enzymes and Coenzymes::Enzymes::Oxidoreductases::Oxidoreductases Acting on CH-NH2 Group Donors::Amino Acid Oxidoreductases::Nitric Oxide Synthase
Medical Subject Headings::Phenomena and Processes::Metabolic Phenomena::Metabolism::Oxidative Stress
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Protein Isoforms
Medical Subject Headings::Chemicals and Drugs::Nucleic Acids, Nucleotides, and Nucleosides::Nucleic Acids::RNA::RNA, Messenger
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Transcription Factors::NF-kappa B::Transcription Factor RelA
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Amino Acids::Amino Acids, Cyclic::Amino Acids, Aromatic::Tyrosine
Medical Subject Headings::Anatomy::Nervous System::Central Nervous System::Brain
Medical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Models, Animal::Disease Models, Animal
Medical Subject Headings::Diseases::Congenital, Hereditary, and Neonatal Diseases and Abnormalities::Genetic Diseases, Inborn::Genetic Diseases, X-Linked::Mental Retardation, X-Linked::Fragile X Syndrome
Medical Subject Headings::Check Tags::Male
Medical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Rodentia::Muridae::Murinae::Mice
Medical Subject Headings::Organisms::Eukaryota::Animals::Animal Population Groups::Animals, Genetically Modified::Mice, Transgenic::Mice, Knockout
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Transcription Factors::NF-kappa B
Medical Subject Headings::Chemicals and Drugs::Inorganic Chemicals::Nitrogen Compounds::Nitrogen Oxides::Nitric Oxide
Medical Subject Headings::Chemicals and Drugs::Enzymes and Coenzymes::Enzymes::Oxidoreductases::Oxidoreductases Acting on CH-NH2 Group Donors::Amino Acid Oxidoreductases::Nitric Oxide Synthase
Medical Subject Headings::Phenomena and Processes::Metabolic Phenomena::Metabolism::Oxidative Stress
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Protein Isoforms
Medical Subject Headings::Chemicals and Drugs::Nucleic Acids, Nucleotides, and Nucleosides::Nucleic Acids::RNA::RNA, Messenger
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Transcription Factors::NF-kappa B::Transcription Factor RelA
Medical Subject Headings::Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Amino Acids::Amino Acids, Cyclic::Amino Acids, Aromatic::Tyrosine
DeCS Terms
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
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
CIE Terms
Keywords
Fragile X syndrome, Nitric oxide metabolism, Protein nitration, Nitric oxide synthase, Nuclear factor kappa B, Brain, Mouse model, Oxidative stress
Citation
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