GABAA Receptor 2 Subunit Mutations Linked to Human Epileptic Syndromes Differentially Affect Phasic and Tonic Inhibition

scientific article published on 01 December 2007

GABAA Receptor 2 Subunit Mutations Linked to Human Epileptic Syndromes Differentially Affect Phasic and Tonic Inhibition is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1523/JNEUROSCI.2618-07.2007
P932PMC publication ID6673514
P698PubMed publication ID18094250

P50authorStéphanie BaulacQ47402324
Jean-Marc FritschyQ56978414
Michel BaulacQ114783855
Jean Christophe PoncerQ42791426
P2093author name stringChristel Depienne
Richard Miles
Stéphanie Baulac
Emmanuel Eugène
Eric Le Guern
P2860cites workGABA(A)-receptor-associated protein links GABA(A) receptors and the cytoskeletonQ22008682
Development of a tonic form of synaptic inhibition in rat cerebellar granule cells resulting from persistent activation of GABAA receptorsQ24530653
Truncation of the GABA(A)-receptor gamma2 subunit in a family with generalized epilepsy with febrile seizures plusQ24632952
First genetic evidence of GABA(A) receptor dysfunction in epilepsy: a mutation in the gamma2-subunit geneQ28186139
Mutant GABA(A) receptor gamma2-subunit in childhood absence epilepsy and febrile seizuresQ28186159
A splice-site mutation in GABRG2 associated with childhood absence epilepsy and febrile convulsionsQ28214606
Childhood absence epilepsy and febrile seizures: a family with a GABA(A) receptor mutationQ28217195
Tonically activated GABAA receptors in hippocampal neurons are high-affinity, low-conductance sensors for extracellular GABAQ28218182
GABRD encoding a protein for extra- or peri-synaptic GABAA receptors is a susceptibility locus for generalized epilepsiesQ28259064
Benzodiazepine-insensitive mice generated by targeted disruption of the gamma 2 subunit gene of gamma-aminobutyric acid type A receptorsQ28367959
Multiple and plastic receptors mediate tonic GABAA receptor currents in the hippocampus.Q46776174
The gamma 2 subunit of GABA(A) receptors is required for maintenance of receptors at mature synapses.Q48165507
Postsynaptic clustering of major GABAA receptor subtypes requires the gamma 2 subunit and gephyrinQ48240102
Shunting inhibition modulates neuronal gain during synaptic excitation.Q48313474
Rat and human hippocampal alpha5 subunit-containing gamma-aminobutyric AcidA receptors have alpha5 beta3 gamma2 pharmacological characteristics.Q48357304
Extrasynaptic alphabeta subunit GABAA receptors on rat hippocampal pyramidal neurons.Q48403423
Properties of putative cerebellar gamma-aminobutyric acid A receptor isoformsQ49100043
Modulation of synaptic GABAA receptor function by benzodiazepines in area CA3 of rat hippocampal slice cultures.Q51580768
α5GABAA Receptors Regulate the Intrinsic Excitability of Mouse Hippocampal Pyramidal NeuronsQ60706586
Constitutive endocytosis of GABAA receptors by an association with the adaptin AP2 complex modulates inhibitory synaptic currents in hippocampal neuronsQ73119677
A gamma 2(R43Q) mutation, linked to epilepsy in humans, alters GABAA receptor assembly and modifies subunit composition on the cell surfaceQ79407662
Activated radixin is essential for GABAA receptor alpha5 subunit anchoring at the actin cytoskeletonQ28568588
Variations on an inhibitory theme: phasic and tonic activation of GABA(A) receptorsQ29616809
Distribution of alpha1, alpha4, gamma2, and delta subunits of GABAA receptors in hippocampal granule cellsQ30435142
Cultured Hippocampal Pyramidal Neurons Express Two Kinds of GABAA ReceptorsQ30435443
Neuroactive steroids reduce neuronal excitability by selectively enhancing tonic inhibition mediated by delta subunit-containing GABAA receptorsQ33717685
Homology model of the GABAA receptor examined using Brownian dynamicsQ34190169
Synchronization of neuronal activity in hippocampus by individual GABAergic interneurons.Q34288636
Altered kinetics and benzodiazepine sensitivity of a GABAA receptor subunit mutation [gamma 2(R43Q)] found in human epilepsy.Q34381755
[3H]L-655,708, a novel ligand selective for the benzodiazepine site of GABAA receptors which contain the alpha 5 subunitQ34414902
Synaptic and extrasynaptic gamma -aminobutyric acid type A receptor clusters in rat hippocampal cultures during developmentQ34743113
Experimental febrile seizures are precipitated by a hyperthermia-induced respiratory alkalosisQ35222197
Tonically active GABA A receptors: modulating gain and maintaining the tone.Q35757312
Distinct gamma2 subunit domains mediate clustering and synaptic function of postsynaptic GABAA receptors and gephyrin.Q36509663
The gamma2 subunit of GABA(A) receptors is a substrate for palmitoylation by GODZQ36631283
GABAA-receptor heterogeneity in the adult rat brain: differential regional and cellular distribution of seven major subunitsQ36790682
Expression of functional receptors by the human gamma-aminobutyric acid A gamma 2 subunitQ36854807
Tonic inhibition in mouse hippocampal CA1 pyramidal neurons is mediated by alpha5 subunit-containing gamma-aminobutyric acid type A receptorsQ37074693
Heterogeneity of GABAA-receptors: cell-specific expression, pharmacology, and regulationQ40464777
Why does fever trigger febrile seizures? GABAA receptor gamma2 subunit mutations associated with idiopathic generalized epilepsies have temperature-dependent trafficking deficiencies.Q40484937
The GABAA receptor gamma2 subunit R43Q mutation linked to childhood absence epilepsy and febrile seizures causes retention of alpha1beta2gamma2S receptors in the endoplasmic reticulum.Q40506831
On the mechanism of a mutated and abnormally functioning gamma-aminobutyric acid (A) receptor linked to epilepsyQ40548516
Selective modulation of tonic and phasic inhibitions in dentate gyrus granule cellsQ40644380
Proton sensitivity of the GABA(A) receptor is associated with the receptor subunit compositionQ41205430
The impact of receptor desensitization on fast synaptic transmissionQ41367149
GABA(A) receptors: immunocytochemical distribution of 13 subunits in the adult rat brain.Q42497057
In vivo determination of extracellular concentration of amino acids in the rat hippocampus. A method based on brain dialysis and computerized analysisQ42526175
An asymmetric contribution to gamma-aminobutyric type A receptor function of a conserved lysine within TM2-3 of alpha1, beta2, and gamma2 subunitsQ42683643
Specific subtypes of GABAA receptors mediate phasic and tonic forms of inhibition in hippocampal pyramidal neurons.Q42691157
In vivo microdialysis of amino acid neurotransmitters in the hippocampus in amygdaloid kindled ratQ43451812
Distinct functional and pharmacological properties of tonic and quantal inhibitory postsynaptic currents mediated by gamma-aminobutyric acid(A) receptors in hippocampal neurons.Q43548389
Enforcement of temporal fidelity in pyramidal cells by somatic feed-forward inhibitionQ43703062
Intact sorting, targeting, and clustering of gamma-aminobutyric acid A receptor subtypes in hippocampal neurons in vitroQ43856121
GABAergic innervation organizes synaptic and extrasynaptic GABAA receptor clustering in cultured hippocampal neurons.Q43875570
Cell type- and input-specific differences in the number and subtypes of synaptic GABA(A) receptors in the hippocampus.Q43940149
Two different mechanisms of disinhibition produced by GABAA receptor mutations linked to epilepsy in humans.Q44050048
GABAergic and glutamatergic terminals differentially influence the organization of GABAergic synapses in rat cerebellar granule cells in vitro.Q44122989
GABA uptake regulates cortical excitability via cell type-specific tonic inhibitionQ44393104
Downregulation of the alpha5 subunit of the GABA(A) receptor in the pilocarpine model of temporal lobe epilepsy.Q44551339
Perforated patch-clamp analysis of the passive membrane properties of three classes of hippocampal neuronsQ44730562
Hippocampal network hyperactivity after selective reduction of tonic inhibition in GABA A receptor alpha5 subunit-deficient mice.Q44986641
Clustering of extrasynaptic GABA(A) receptors modulates tonic inhibition in cultured hippocampal neuronsQ45022863
Tonic and spillover inhibition of granule cells control information flow through cerebellar cortexQ45713983
The epilepsy mutation, gamma2(R43Q) disrupts a highly conserved inter-subunit contact site, perturbing the biogenesis of GABAA receptorsQ46467962
Low concentrations of penicillin reveal rhythmic, synchronous synaptic potentials in hippocampal sliceQ46695488
P433issue51
P407language of work or nameEnglishQ1860
P304page(s)14108-14116
P577publication date2007-12-01
P1433published inJournal of NeuroscienceQ1709864
P1476titleGABA(A) receptor gamma 2 subunit mutations linked to human epileptic syndromes differentially affect phasic and tonic inhibition
P478volume27

Reverse relations

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