ArticleCerebral cortex (New York, N.Y. : 1991)2009
Limbic epileptogenesis in a mouse model of fragile X syndrome.
Article in Cerebral cortex (New York, N.Y. : 1991), 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
19 citing papers in PubMed, 33 citations in OpenAlex.
- Bioisostere-Driven Discovery of SePP: A Selenium-Containing Polypharmacological Agent Relevant to Fragile X Syndrome.Journal of medicinal chemistry · 2026Article
- Compensatory Regulation of Excitation/Inhibition Balance in the Ventral Hippocampus: Insights from Fragile X Syndrome.Biology · 2025Review
- Rescue of sharp wave-ripples and prevention of network hyperexcitability in the ventral but not the dorsal hippocampus of a rat model of fragile X syndrome.Frontiers in cellular neuroscience · 2023Article
- Spontaneous seizures in adult Fmr1 knockout mice: FVB.129P2-Pde6bEpilepsy research · 2022Article
- Hyperexcitability and Homeostasis in Fragile X Syndrome.Frontiers in molecular neuroscience · 2021Article
- Differential roles of α-, β-, and γ-actin in axon growth and collateral branch formation in motoneurons.The Journal of cell biology · 2017Article
- Fragile X syndrome as a rare disease in China - Therapeutic challenges and opportunities.Intractable & rare diseases research · 2015Review
- Stem cells and modeling of autism spectrum disorders.Experimental neurology · 2014Review
- From FMRP function to potential therapies for fragile X syndrome.Neurochemical research · 2014Review
- Involvement of Rho-family GTPases in axon branching.Small GTPases · 2014Review
- Article
- Fragile X syndrome and targeted treatment trials.Results and problems in cell differentiation · 2012Review
- Therapeutic strategies in fragile X syndrome: dysregulated mGluR signaling and beyond.Neuropsychopharmacology : official publication of the American College of Neuropsychopharmacology · 2012Review
- Annual Research Review: Transgenic mouse models of childhood-onset psychiatric disorders.Journal of child psychology and psychiatry, and allied disciplines · 2011Review
- Genetic-background modulation of core and variable autistic-like symptoms in Fmr1 knock-out mice.PloS one · 2011Article
- Hypersensitivity to mGluR5 and ERK1/2 leads to excessive protein synthesis in the hippocampus of a mouse model of fragile X syndrome.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2010Article
- Emerging pharmacotherapies for neurodevelopmental disorders.Journal of developmental and behavioral pediatrics : JDBP · 2010Review
- Fragile X mental retardation protein in the driver's seat.Cerebral cortex (New York, N.Y. : 1991) · 2009Article
- Origins of epilepsy in fragile X syndrome.Epilepsy currentsArticle
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Authors and funding
6 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Fragile X syndrome (FXS), caused by silencing of the Fmr1 gene, is the most common form of inherited mental retardation. Epilepsy is reported to occur in 20-25% of individuals with FXS. However, no overall increased excitability has been reported in Fmr1 knockout (KO) mice, except for increased sensitivity to auditory stimulation. Here, we report that kindling increased the expressions of Fmr1 mRNA and protein in the forebrain of wild-type (WT) mice. Kindling development was dramatically accelerated in Fmr1 KO mice, and Fmr1 KO mice also displayed prolonged electrographic seizures during kindling and more severe mossy fiber sprouting after kindling. The accelerated rate of kindling was partially repressed by inhibiting N-methyl-D-aspartic acid receptor (NMDAR) with MK-801 or mGluR5 receptor with 2-methyl-6-(phenylethynyl)-pyridine (MPEP). The rate of kindling development in WT was not effected by MPEP, however, suggesting that FMRP normally suppresses epileptogenic signaling downstream of metabolic glutamate receptors. Our findings reveal that FMRP plays a critical role in suppressing limbic epileptogenesis and predict that the enhanced susceptibility of patients with FXS to epilepsy is a direct consequence of the loss of an important homeostatic factor that mitigates vulnerability to excessive neuronal excitation.
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Registered trials
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.