RGD Reference Report - Sensory hypo-excitability in a rat model of fetal development in Fragile X Syndrome. - Rat Genome Database

Send us a Message
Submit Data |  Help |  Video Tutorials |  News |  Publications |  Download |  REST API |  Citing RGD |  Contact   
Search RGD Grant Resources Citing RGD About Us Contact Us
Genes Variants Community Projects QTLs Strains Markers Genome Information Ontologies Cell Lines References Download Submit Data
OntoMate (Literature Search) JBrowse (Genome Browser) Synteny Browser (VCMap) Variant Visualizer Multi-Ontology Enrichment (MOET) Gene-Ortholog Location Finder (GOLF) InterViewer (Protein-Protein Interactions) PhenoMiner (Quatitative Phenotypes) Gene Annotator OLGA (Gene List Generator) AllianceMine GViewer (Genome Viewer)
Aging & Age-Related Disease Cancer & Neoplastic Disease Cardiovascular Disease Coronavirus Disease Developmental Disease Diabetes Hematologic Disease Immune & Inflammatory Disease Infectious Disease Liver Disease Neurological Disease Obesity & Metabolic Syndrome Renal Disease Respiratory Disease Sensory Organ Disease
Find Models Genetic Models Autism Models Rat PhenoMiner (Quantitative Phenotypes) Chinchilla PhenoMiner Expected Ranges (Quantitative Phenotype) PhenoMiner Term Comparison Hybrid Rat Diversity Panel Phenotypes Phenotypes in Other Animal Models Animal Husbandry Strain Medical Records Phylogenetics Strain Availability Calendar Rats 101 Submissions Photo Archive
Pathways
Rat Community Forum Directory of Rat Laboratories Video Tutorials News RGD Publications RGD Presentations Archive Nomenclature Guidelines Resource Links Laboratory Resources Employment Resources
Advanced Search (OLGA)

Sensory hypo-excitability in a rat model of fetal development in Fragile X Syndrome.

Authors: Berzhanskaya, Julia  Phillips, Marnie A  Shen, Jing  Colonnese, Matthew T 
Citation: Berzhanskaya J, etal., Sci Rep. 2016 Jul 28;6:30769. doi: 10.1038/srep30769.
RGD ID: 38548926
Pubmed: PMID:27465362   (View Abstract at PubMed)
PMCID: PMC4964352   (View Article at PubMed Central)
DOI: DOI:10.1038/srep30769   (Journal Full-text)

Fragile X syndrome (FXS) is characterized by sensory hyper-sensitivity, and animal models suggest that neuronal hyper-excitability contributes to this phenotype. To understand how sensory dysfunction develops in FXS, we used the rat model (FMR-KO) to quantify the maturation of cortical visual responses from the onset of responsiveness prior to eye-opening, through age equivalents of human juveniles. Rather than hyper-excitability, visual responses before eye-opening had reduced spike rates and an absence of early gamma oscillations, a marker for normal thalamic function at this age. Despite early hypo-excitability, the developmental trajectory of visual responses in FMR-KO rats was normal, and showed the expected loss of visually evoked bursting at the same age as wild-type, two days before eye-opening. At later ages, during the third and fourth post-natal weeks, signs of mild hyper-excitability emerged. These included an increase in the visually-evoked firing of regular spiking, presumptive excitatory, neurons, and a reduced firing of fast-spiking, presumptive inhibitory, neurons. Our results show that early network changes in the FMR-KO rat arise at ages equivalent to fetal humans and have consequences for excitability that are opposite those found in adults. This suggests identification and treatment should begin early, and be tailored in an age-appropriate manner.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
FMR1Humanfragile X syndrome  ISORGD:2623DNA:deletion:intron 7, exon 8:RGD 
Fmr1Ratfragile X syndrome  IMP DNA:deletion:intron 7, exon 8:RGD 
Fmr1Mousefragile X syndrome  ISORGD:2623DNA:deletion:intron 7, exon 8:RGD 
Fmr1em1SageRatfragile X syndrome  IMP DNA:deletion:intron 7, exon 8:RGD 
SD-Fmr1em1SageRatfragile X syndrome MODELIMP compared to SD control;DNA:deletion:intron 7, exon 8:RGD 

Gene Ontology Annotations    Click to see Annotation Detail View

Biological Process

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
Fmr1Ratregulation of neuronal action potential  IMP  RGD 

Phenotype Annotations    Click to see Annotation Detail View

Mammalian Phenotype

Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
Fmr1Ratabnormal visual evoked potential  IMP DNA:deletion:intron 7, exon 8:RGD 
Fmr1em1SageRatabnormal visual evoked potential  IMP DNA:deletion:intron 7, exon 8:RGD 
SD-Fmr1em1SageRatabnormal visual evoked potential  IMP compared to SD control, DNA:deletion:intron 7, exon 8:RGD 
Objects Annotated

Genes (Rattus norvegicus)
Fmr1  (fragile X messenger ribonucleoprotein 1)
Fmr1em1Sage  (FMRP translational regulator 1; zinc finger nuclease induced mutant 1, Sigma Advanced Genetic Engineering Labs)

Genes (Mus musculus)
Fmr1  (fragile X messenger ribonucleoprotein 1)

Genes (Homo sapiens)
FMR1  (fragile X messenger ribonucleoprotein 1)

Strains
SD-Fmr1em1Sage  (NA)


Additional Information