ArticleEpilepsia2012
Abnormal dendrite and spine morphology in primary visual cortex in the CGG knock-in mouse model of the fragile X premutation.
Article in Epilepsia, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.
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Who cites it
36 citing papers in PubMed, 59 citations in OpenAlex.
- Article
- Visual function and autism spectrum disorder.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Stimulation of the medial septum diagonal band of broca rescues learning and memory deficits iniScience · 2026Article
- Antisense oligonucleotides to KIF1A polymorphisms expand targets and rescue patient-derived neurons in vitro.Nature communications · 2026Article
- Hippocampal-prefrontal functional neural networks in a rat model of fragile X syndrome are poorly organized with limited resiliency.Scientific reports · 2025Article
- A Comprehensive Overview of the Role of Visual Cortex Malfunction in Depressive Disorders: Opportunities and Challenges.Neuroscience bulletin · 2023Review
- Gene×environment interactions in autism spectrum disorders.Current topics in developmental biology · 2023Review
- Restoration of FMRP expression in adult V1 neurons rescues visual deficits in a mouse model of fragile X syndrome.Protein & cell · 2022Article
- Sex and Genotype Modulate the Dendritic Effects of Developmental Exposure to a Human-Relevant Polychlorinated Biphenyls Mixture in the Juvenile Mouse.Frontiers in neuroscience · 2021Article
- Role of age and neuroinflammation in the mechanism of cognitive deficits in sickle cell disease.Experimental biology and medicine (Maywood, N.J.) · 2021Article
- Cellular localization of the FMRP in rat retina.Bioscience reports · 2020Article
- A potential role of fatty acid binding protein 4 in the pathophysiology of autism spectrum disorder.Brain communications · 2020Article
- Reduction of Fmr1 mRNA Levels Rescues Pathological Features in Cortical Neurons in a Model of FXTAS.Molecular therapy. Nucleic acids · 2019Article
- Genetic mutations in CaGenes, brain, and behavior · 2019Article
- The Autism Protein Ube3A/E6AP Remodels Neuronal Dendritic Arborization via Caspase-Dependent Microtubule Destabilization.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2018Article
- Early Retinal Defects inFrontiers in cellular neuroscience · 2018Article
- Functional roles of p120ctn family of proteins in central neurons.Seminars in cell & developmental biology · 2017Review
- Calcium dysregulation and Cdk5-ATM pathway involved in a mouse model of fragile X-associated tremor/ataxia syndrome.Human molecular genetics · 2017Article
- Dendrite and spine modifications in autism and related neurodevelopmental disorders in patients and animal models.Developmental neurobiology · 2017Review
- Lens Biology is a Dimension of Neurobiology.Neurochemical research · 2017Review
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
The fragile X mental retardation 1 gene (Fmr1) is polymorphic for CGG trinucleotide repeat number in the 5'-untranslated region, with repeat lengths <45 associated with typical development and repeat lengths >200 resulting in hypermethylation and transcriptional silencing of the gene and mental retardation in the fragile X Syndrome (FXS). Individuals with CGG repeat expansions between 55 and 200 are carriers of the fragile X premutation (PM). PM carriers show a phenotype that can include anxiety, depression, social phobia, and memory deficits. They are also at risk for developing fragile X-associated tremor/ataxia syndrome (FXTAS), a late onset neurodegenerative disorder characterized by tremor, ataxia, cognitive impairment, and neuropathologic features including intranuclear inclusions in neurons and astrocytes, loss of Purkinje cells, and white matter disease. However, very little is known about dendritic morphology in PM or in FXTAS. Therefore, we carried out a Golgi study of dendritic complexity and dendritic spine morphology in layer II/III pyramidal neurons in primary visual cortex in a knock-in (KI) mouse model of the PM. These CGG KI mice carry an expanded CGG trinucleotide repeat on Fmr1, and model many features of the PM and FXTAS. Compared to wild-type (WT) mice, CGG KI mice showed fewer dendritic branches proximal to the soma, reduced total dendritic length, and a higher frequency of longer dendritic spines. The distribution of morphologic spine types (e.g., stubby, mushroom, filopodial) did not differ between WT and KI mice. These findings demonstrate that synaptic circuitry is abnormal in visual cortex of mice used to model the PM, and suggest that such changes may underlie neurologic features found in individuals carrying the PM as well as in individuals with FXTAS.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.