Fragile X syndrome: the GABAergic system and circuit dysfunction

scientific article published on 21 September 2011

Fragile X syndrome: the GABAergic system and circuit dysfunction is …
instance of (P31):
review articleQ7318358
scholarly articleQ13442814

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P356DOI10.1159/000329420
P932PMC publication ID3254035
P698PubMed publication ID21934270
P5875ResearchGate publication ID51658136

P2093author name stringMolly M Huntsman
Scott M Paluszkiewicz
Brandon S Martin
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Fragile X syndrome: loss of local mRNA regulation alters synaptic development and functionQ24608468
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Abnormal dendritic spines in fragile X knockout mice: maturation and pruning deficitsQ24657867
Pharmacological characterization of a novel cell line expressing human alpha(4)beta(3)delta GABA(A) receptorsQ24673026
Activity-dependent validation of excitatory versus inhibitory synapses by neuroligin-1 versus neuroligin-2Q26269865
Increased anxiety-like behavior in mice lacking the inhibitory synapse cell adhesion molecule neuroligin 2Q26269913
Common circuit defect of excitatory-inhibitory balance in mouse models of autismQ26269924
Neuroligin-2 deletion selectively decreases inhibitory synaptic transmission originating from fast-spiking but not from somatostatin-positive interneuronsQ26269926
Downregulation of tonic GABAergic inhibition in a mouse model of fragile X syndromeQ27303466
Immunolocalization of mGluR1alpha in specific populations of local circuit neurons in the cerebral cortexQ28140990
From mRNP trafficking to spine dysmorphogenesis: the roots of fragile X syndromeQ28247567
Neurosteroids: endogenous regulators of the GABA(A) receptorQ28256904
Neuroligin 2 drives postsynaptic assembly at perisomatic inhibitory synapses through gephyrin and collybistinQ28258590
Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndromeQ28273791
The K+/Cl- co-transporter KCC2 renders GABA hyperpolarizing during neuronal maturationQ28296169
Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomesQ28303541
Synaptic imbalance, stereotypies, and impaired social interactions in mice with altered neuroligin 2 expressionQ28564770
Cl- uptake promoting depolarizing GABA actions in immature rat neocortical neurones is mediated by NKCC1Q28566006
Control of excitatory and inhibitory synapse formation by neuroliginsQ28568459
Neonatal development of the rat visual cortex: synaptic function of GABAA receptor alpha subunitsQ28574932
Neuroligin 2 is exclusively localized to inhibitory synapsesQ28576687
Developmentally-programmed FMRP expression in oligodendrocytes: a potential role of FMRP in regulating translation in oligodendroglia progenitorsQ28580499
Independent assembly and subcellular targeting of GABA(A)-receptor subtypes demonstrated in mouse hippocampal and olfactory neurons in vivoQ28586363
The FXG: a presynaptic fragile X granule expressed in a subset of developing brain circuitsQ28588310
Interneurons of the neocortical inhibitory systemQ29547509
Model of autism: increased ratio of excitation/inhibition in key neural systemsQ29547530
Cortical inhibitory neurons and schizophreniaQ29547683
Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypesQ29616326
Postnatal Neurodevelopmental Disorders: Meeting at the Synapse?Q29616332
Fragile X mental retardation protein targets G quartet mRNAs important for neuronal functionQ29616372
Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndromeQ29616495
Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP).Q37411441
The developmental integration of cortical interneurons into a functional networkQ37475593
Amygdala inhibitory circuits and the control of fear memoryQ37531107
Fragile X mental retardation protein control of neuronal mRNA metabolism: Insights into mRNA stabilityQ37616726
GABAB receptor coupling to G-proteins and ion channelsQ37775207
The FMR-1 protein is cytoplasmic, most abundant in neurons and appears normal in carriers of a fragile X premutationQ38317052
Expression of FMR1, FXR1, and FXR2 genes in human prenatal tissuesQ38321701
Evidence for social anxiety and impaired social cognition in a mouse model of fragile X syndromeQ38501407
Open-label riluzole in fragile X syndromeQ39819390
Molecular mechanisms of interaction between the neuroprotective substance riluzole and GABA(A)-receptorsQ39985079
GAD67-mediated GABA synthesis and signaling regulate inhibitory synaptic innervation in the visual cortexQ40097657
Early defects of GABAergic synapses in the brain stem of a MeCP2 mouse model of Rett syndrome.Q40151344
A physiological role for GABAB receptors and the effects of baclofen in the mammalian central nervous systemQ40929779
Fragile-X syndrome: a particular epileptogenic EEG patternQ41433374
Temporal modulation of GABA(A) receptor subunit gene expression in developing monkey cerebral cortexQ41676923
Highly specific neuron loss preserves lateral inhibitory circuits in the dentate gyrus of kainate-induced epileptic ratsQ41697748
GABA(A) receptor-mediated miniature postsynaptic currents and alpha-subunit expression in developing cortical neuronsQ41708997
Nucleus-specific differences in GABA(A)-receptor-mediated inhibition are enhanced during thalamic developmentQ41714103
Critical period plasticity is disrupted in the barrel cortex of FMR1 knockout mice.Q41844282
Fragile X mental retardation protein regulates the levels of scaffold proteins and glutamate receptors in postsynaptic densitiesQ42042937
Sorting of striatal and cortical interneurons regulated by semaphorin-neuropilin interactionsQ42508121
Major defects in neocortical GABAergic inhibitory circuits in mice lacking the fragile X mental retardation proteinQ42875155
Early developmental alterations in GABAergic protein expression in fragile X knockout miceQ43063055
Origins of epilepsy in fragile X syndromeQ43067748
Altered neuroligin expression is involved in social deficits in a mouse model of the fragile X syndromeQ43237114
Diverse types of interneurons generate thalamus-evoked feedforward inhibition in the mouse barrel cortex.Q43578195
GABA(A) receptor alpha1 subunit deletion prevents developmental changes of inhibitory synaptic currents in cerebellar neurons.Q43581695
GABA(B) receptors mediate motility signals for migrating embryonic cortical cellsQ43679920
Characterization of tiagabine (NO-328), a new potent and selective GABA uptake inhibitorQ43838084
Reduced cortical synaptic plasticity and GluR1 expression associated with fragile X mental retardation protein deficiency.Q43892381
Cell type- and input-specific differences in the number and subtypes of synaptic GABA(A) receptors in the hippocampus.Q43940149
Early sequential formation of functional GABA(A) and glutamatergic synapses on CA1 interneurons of the rat foetal hippocampusQ44095430
Evidence that Sema3A and Sema3F regulate the migration of GABAergic neurons in the developing neocortexQ44231704
Blockade of GABA(B) receptors alters the tangential migration of cortical neurons.Q44539346
GABAA receptor maturation in relation to eye opening in the rat visual cortexQ44762478
The distribution of thirteen GABAA receptor subunit mRNAs in the rat brain. III. Embryonic and postnatal developmentQ44767968
Age- and region-specific imbalances of basal amino acids and monoamine metabolism in limbic regions of female Fmr1 knock-out miceQ44843160
Hippocampal network hyperactivity after selective reduction of tonic inhibition in GABA A receptor alpha5 subunit-deficient mice.Q44986641
Role of class 3 semaphorins in the development and maturation of the septohippocampal pathway.Q45073794
Experience and activity-dependent maturation of perisomatic GABAergic innervation in primary visual cortex during a postnatal critical period.Q45127985
Time-matched pre- and postsynaptic changes of GABAergic synaptic transmission in the developing mouse superior colliculusQ45231919
Slow kinetics of miniature IPSCs during early postnatal development in granule cells of the dentate gyrus.Q45974379
Increased GABA(B) receptor-mediated signaling reduces the susceptibility of fragile X knockout mice to audiogenic seizuresQ46058214
Developmental expression of GABA(A) receptor subunit and GAD genes in mouse somatosensory barrel cortexQ46081132
Taurine improves congestive functions in a mouse model of fragile X syndromeQ46111209
Expression of the GABAergic system in animal models for fragile X syndrome and fragile X associated tremor/ataxia syndrome (FXTAS).Q46205774
Selective resistance of taurine-fed mice to isoniazide-potentiated seizures: in vivo functional test for the activity of glutamic acid decarboxylase.Q46410783
Developmental profile of GABAA-mediated synaptic transmission in pyramidal cells of the somatosensory cortexQ46437563
A noncanonical release of GABA and glutamate modulates neuronal migration.Q46486119
Disruption of postsynaptic GABA receptor clusters leads to decreased GABAergic innervation of pyramidal neuronsQ46527450
Suppression of two major Fragile X Syndrome mouse model phenotypes by the mGluR5 antagonist MPEP.Q46626541
Prolonged epileptiform discharges induced by altered group I metabotropic glutamate receptor-mediated synaptic responses in hippocampal slices of a fragile X mouse model.Q46681406
Defined types of cortical interneurone structure space and spike timing in the hippocampusQ35946394
Modulation of neocortical interneurons: extrinsic influences and exercises in self-controlQ36247696
Attenuated sensitivity to neuroactive steroids in gamma-aminobutyrate type A receptor delta subunit knockout mice.Q36562979
Fragile X syndrome and autism at the intersection of genetic and neural networksQ36603922
Neuroanatomy of fragile X syndrome: the temporal lobeQ36734713
Switch in the expression of rat GABAA-receptor subtypes during postnatal development: an immunohistochemical studyQ36739893
Gephyrin clustering is required for the stability of GABAergic synapsesQ36766163
Refining the roles of GABAergic signaling during neural circuit formationQ36859347
Pyramidal cells of the rat basolateral amygdala: synaptology and innervation by parvalbumin-immunoreactive interneuronsQ36924433
Quantitative proteomic analysis of primary neurons reveals diverse changes in synaptic protein content in fmr1 knockout miceQ36926795
Advances in behavioral genetics: mouse models of autismQ36937876
Imbalance of neocortical excitation and inhibition and altered UP states reflect network hyperexcitability in the mouse model of fragile X syndromeQ36978553
Activation of GABAA receptors: views from outside the synaptic cleft.Q37022454
Role for metabotropic glutamate receptor 5 (mGluR5) in the pathogenesis of fragile X syndromeQ37059491
Frontostriatal deficits in fragile X syndrome: relation to FMR1 gene expressionQ37074462
Multiple modes of network homeostasis in visual cortical layer 2/3.Q37127176
Social approach in genetically engineered mouse lines relevant to autismQ37138180
Differential metabotropic glutamate receptor expression and modulation in two neocortical inhibitory networksQ37190458
GABA regulates excitatory synapse formation in the neocortex via NMDA receptor activationQ37198498
Axonal GABAA receptors.Q37240087
Differential synaptic localization of two major gamma-aminobutyric acid type A receptor alpha subunits on hippocampal pyramidal cellsQ37275701
Defining the role of GABA in cortical development.Q37372212
Activity-dependent development of inhibitory synapses and innervation pattern: role of GABA signalling and beyond.Q37383902
Expression profiling suggests underexpression of the GABA(A) receptor subunit delta in the fragile X knockout mouse modelQ46731641
Abnormal striatal GABA transmission in the mouse model for the fragile X syndrome.Q46889350
Expression of distinct alpha subunits of GABAA receptor regulates inhibitory synaptic strengthQ47717822
Riluzole, a novel antiglutamate, blocks GABA uptake by striatal synaptosomesQ48123517
Epilepsy and EEG findings in males with fragile X syndrome.Q48135370
Fronto-striatal dysfunction and potential compensatory mechanisms in male adolescents with fragile X syndromeQ48201398
Nucleus basalis magnocellularis and hippocampus are the major sites of FMR-1 expression in the human fetal brainQ48275073
Two dynamically distinct inhibitory networks in layer 4 of the neocortexQ48276407
Somatosensory cortical barrel dendritic abnormalities in a mouse model of the fragile X mental retardation syndromeQ48337615
Tissue specific expression of FMR-1 provides evidence for a functional role in fragile X syndromeQ48351859
Postsynaptic targets of somatostatin-containing interneurons in the rat basolateral amygdalaQ48362067
RNA cargoes associating with FMRP reveal deficits in cellular functioning in Fmr1 null miceQ48392342
Decreased expression of the GABAA receptor in fragile X syndromeQ48394848
Potentiation of cortical inhibition by visual deprivation.Q48438986
Developmental changes in KCC1, KCC2, and NKCC1 mRNA expressions in the rat brain.Q48455129
Short-term treatment with gaboxadol improves sleep maintenance and enhances slow wave sleep in adult patients with primary insomnia.Q48461687
Effect of short-term treatment with gaboxadol on sleep maintenance and initiation in patients with primary insomnia.Q48472265
Early postnatal plasticity in neocortex of Fmr1 knockout mice.Q48478142
Inhibitory synaptogenesis in mouse somatosensory cortex.Q48579158
Expression of group one metabotropic glutamate receptor subunit mRNAs in neurochemically identified neurons in the rat neostriatum, neocortex, and hippocampus.Q48615671
GABAergic cell subtypes and their synaptic connections in rat frontal cortex.Q48632270
Quantitative aspects of synaptogenesis in the rat barrel field cortex with special reference to GABA circuitry.Q48914786
Mice lacking the major adult GABAA receptor subtype have normal number of synapses, but retain juvenile IPSC kinetics until adulthood.Q48975128
Fragile X mouse: strain effects of knockout phenotype and evidence suggesting deficient amygdala function.Q51083891
Decreased GABA(A) receptor expression in the seizure-prone fragile X mouse.Q51932364
Epilepsy in fragile X syndrome.Q51952378
Electrodermal responses to sensory stimuli in individuals with fragile X syndrome: a preliminary report.Q51985541
Depolarizing GABAergic conductances regulate the balance of excitation to inhibition in the developing retinotectal circuit in vivo.Q52018171
Neurodevelopmental effects of the FMR-1 full mutation in humans.Q52019051
A double-blind, placebo-controlled study of vigabatrin three g/day in patients with uncontrolled complex partial seizures. Vigabatrin Protocol 024 Investigative CohortQ57544660
Two networks of electrically coupled inhibitory neurons in neocortexQ57860911
A network of fast-spiking cells in the neocortex connected by electrical synapsesQ59090645
The Establishment of GABAergic and Glutamatergic Synapses on CA1 Pyramidal Neurons is Sequential and Correlates with the Development of the Apical DendriteQ63433206
Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosisQ73206517
Immunocytochemical and biochemical characterization of FMRP, FXR1P, and FXR2P in the mouseQ74060519
GABA receptor antagonists modulate postmitotic cell migration in slice cultures of embryonic rat cortexQ74303565
Salient features of synaptic organisation in the cerebral cortexQ74746755
The fragile X syndromeQ74809876
Autism spectrum disorder in Fragile X syndrome: differential contribution of adaptive socialization and social withdrawalQ80103577
Variations on an inhibitory theme: phasic and tonic activation of GABA(A) receptorsQ29616809
Excitatory actions of gaba during development: the nature of the nurtureQ29617435
Parvalbumin neurons and gamma rhythms enhance cortical circuit performanceQ29619163
Long-term sensory deprivation selectively rearranges functional inhibitory circuits in mouse barrel cortex.Q30488660
Defective GABAergic neurotransmission and pharmacological rescue of neuronal hyperexcitability in the amygdala in a mouse model of fragile X syndromeQ30496828
Dendritic but not somatic GABAergic inhibition is decreased in experimental epilepsy.Q31793881
Input-dependent synaptic targeting of alpha(2)-subunit-containing GABA(A) receptors in synapses of hippocampal pyramidal cells of the rat.Q31900249
Abnormal dendritic spine characteristics in the temporal and visual cortices of patients with fragile-X syndrome: a quantitative examination.Q32063419
Identification of small molecules rescuing fragile X syndrome phenotypes in DrosophilaQ33323032
An anatomical substrate for experience-dependent plasticity of the rat barrel field cortexQ33727555
Differential modulation of proliferation in the neocortical ventricular and subventricular zonesQ33911054
Characterization and reversal of synaptic defects in the amygdala in a mouse model of fragile X syndromeQ33953276
Characterization of vigabatrin‐associated optic atrophyQ33972736
The emotional brain, fear, and the amygdalaQ33972788
Enhanced endocannabinoid signaling elevates neuronal excitability in fragile X syndromeQ34003224
Neurosteroids and epilepsyQ34099166
Endocannabinoid signaling in the brainQ34125520
Seizures in fragile X syndrome: characteristics and comorbid diagnosesQ34143566
Subunit composition, distribution and function of GABA(A) receptor subtypesQ34143787
Epigenetic modification of the FMR1 gene in fragile X syndrome is associated with differential response to the mGluR5 antagonist AFQ056.Q34157712
The role of GABA in the pathophysiology and treatment of anxiety disorders.Q34319322
Pharmacological characterization of GABAA receptors in taurine-fed mice.Q34364433
Neuro-endocrine basis for altered plasma glucose homeostasis in the Fragile X mouseQ34364470
GABA and glutamate depolarize cortical progenitor cells and inhibit DNA synthesisQ34400922
RNA targets of the fragile X protein.Q34456641
GABA-based therapeutic approaches: GABAA receptor subtype functionsQ34479365
The diversity of GABAA receptors. Pharmacological and electrophysiological properties of GABAA channel subtypesQ34481002
MeCP2 dysfunction in Rett syndrome and related disordersQ34519177
Abnormal mGlu 5 receptor/endocannabinoid coupling in mice lacking FMRP and BC1 RNA.Q34660995
Differential response of cortical plate and ventricular zone cells to GABA as a migration stimulus.Q34750391
Circuit and plasticity defects in the developing somatosensory cortex of FMR1 knock-out mice.Q34778657
Formation and plasticity of GABAergic synapses: physiological mechanisms and pathophysiological implications.Q35143354
Tonically active GABA A receptors: modulating gain and maintaining the tone.Q35757312
Molecular structure and physiological functions of GABA(B) receptorsQ35842984
Absence of metabotropic glutamate receptor-mediated plasticity in the neocortex of fragile X mice.Q35844502
P433issue5
P921main subjectfragile X syndromeQ221472
P304page(s)349-364
P577publication date2011-09-21
P1433published inDevelopmental NeuroscienceQ15763713
P1476titleFragile X syndrome: the GABAergic system and circuit dysfunction
P478volume33

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