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NOEBELS, JEFFREY

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Item Type	Name
Academic Article	Mutational analysis of inherited epilepsies.
Academic Article	Genetic epilepsy model derived from common inbred mouse strains.
Academic Article	Molecular characterization of a high-affinity mouse glutamate transporter.
Academic Article	Analysis of voltage-gated and synaptic conductances contributing to network excitability defects in the mutant mouse tottering.
Academic Article	Aberrant expression of neuropeptide Y in hippocampal mossy fibers in the absence of local cell injury following the onset of spike-wave synchronization.
Academic Article	Genetic mapping and evaluation of candidate genes for spasmodic, a neurological mouse mutation with abnormal startle response.
Academic Article	Glutamate transporter mRNA expression in proliferative zones of the developing and adult murine CNS.
Academic Article	Hypomyelination alters K+ channel expression in mouse mutants shiverer and Trembler.
Academic Article	Increased excitability and inward rectification in layer V cortical pyramidal neurons in the epileptic mutant mouse Stargazer.
Academic Article	A candidate model for Angelman syndrome in the mouse.
Academic Article	Molecular and cellular plasticity in developing epileptic brain.
Academic Article	Upregulation of L-type Ca2+ channels in reactive astrocytes after brain injury, hypomyelination, and ischemia.
Academic Article	Overexpression of a Shaker-type potassium channel in mammalian central nervous system dysregulates native potassium channel gene expression.
Academic Article	beta subunit reshuffling modifies N- and P/Q-type Ca2+ channel subunit compositions in lethargic mouse brain.
Academic Article	Loss of BETA2/NeuroD leads to malformation of the dentate gyrus and epilepsy.
Academic Article	Ion channels and epilepsy in man and mouse.
Academic Article	Progressive ataxia, myoclonic epilepsy and cerebellar apoptosis in cystatin B-deficient mice.
Academic Article	Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation.
Academic Article	Rocker is a new variant of the voltage-dependent calcium channel gene Cacna1a.
Academic Article	Absence of hippocampal mossy fiber sprouting in transgenic mice overexpressing brain-derived neurotrophic factor.
Academic Article	Presynaptic Ca2+ channels and neurotransmitter release at the terminal of a mouse cortical neuron.
Academic Article	Voltage-dependent calcium channel mutations in neurological disease.
Academic Article	A burst-dependent hippocampal excitability defect elicited by potassium at the developmental onset of spike-wave seizures in the Tottering mutant.
Academic Article	Presynaptic Ca(2+) influx at a mouse central synapse with Ca(2+) channel subunit mutations.
Academic Article	Genetic localization of the Ca2+ channel gene CACNG2 near SCA10 on chromosome 22q13.
Academic Article	Sodium/hydrogen exchanger gene defect in slow-wave epilepsy mutant mice.
Academic Article	Elevated expression of type II Na+ channels in hypomyelinated axons of shiverer mouse brain.
Academic Article	Genetic disruption of cortical interneuron development causes region- and GABA cell type-specific deficits, epilepsy, and behavioral dysfunction.
Academic Article	Calcium channel defects in models of inherited generalized epilepsy.
Academic Article	Impaired fast-spiking, suppressed cortical inhibition, and increased susceptibility to seizures in mice lacking Kv3.2 K+ channel proteins.
Academic Article	Mutation in AP-3 delta in the mocha mouse links endosomal transport to storage deficiency in platelets, melanosomes, and synaptic vesicles.
Academic Article	Single-gene models of epilepsy.
Academic Article	Mice lacking sodium channel beta1 subunits display defects in neuronal excitability, sodium channel expression, and nodal architecture.
Academic Article	Mice lacking Dlx1 show subtype-specific loss of interneurons, reduced inhibition and epilepsy.
Academic Article	BK channel beta4 subunit reduces dentate gyrus excitability and protects against temporal lobe seizures.
Academic Article	Topiramate alters excitatory synaptic transmission in mouse hippocampus.
Academic Article	Mice with truncated MeCP2 recapitulate many Rett syndrome features and display hyperacetylation of histone H3.
Academic Article	Aberrant excitatory neuronal activity and compensatory remodeling of inhibitory hippocampal circuits in mouse models of Alzheimer's disease.
Academic Article	Elevated thalamic low-voltage-activated currents precede the onset of absence epilepsy in the SNAP25-deficient mouse mutant coloboma.
Academic Article	Masking epilepsy by combining two epilepsy genes.
Academic Article	Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3.
Academic Article	Neuronal LRP1 functionally associates with postsynaptic proteins and is required for normal motor function in mice.
Academic Article	T-type calcium channels: from discovery to channelopathies, 25 years of research.
Academic Article	Genetic mouse models of essential tremor: are they essential?
Academic Article	ADPEAF mutations reduce levels of secreted LGI1, a putative tumor suppressor protein linked to epilepsy.
Academic Article	Visualization of transmitter release with zinc fluorescence detection at the mouse hippocampal mossy fibre synapse.
Academic Article	Mild overexpression of MeCP2 causes a progressive neurological disorder in mice.
Academic Article	Arc regulates spine morphology and maintains network stability in vivo.
Academic Article	Knockout of Zn transporters Zip-1 and Zip-3 attenuates seizure-induced CA1 neurodegeneration.
Academic Article	Ablation of steroid receptor coactivator-3 resembles the human CACT metabolic myopathy.
Academic Article	Interneuron, interrupted: molecular pathogenesis of ARX mutations and X-linked infantile spasms.
Academic Article	Transcompartmental reversal of single fibre hyperexcitability in juxtaparanodal Kv1.1-deficient vagus nerve axons by activation of nodal KCNQ channels.
Academic Article	A mouse model for Glut-1 haploinsufficiency.
Academic Article	Genetic and phenotypic heterogeneity of inherited spike-wave epilepsy: two mutant gene loci with independent cerebral excitability defects.
Academic Article	Exocytosis of vesicular zinc reveals persistent depression of neurotransmitter release during metabotropic glutamate receptor long-term depression at the hippocampal CA3-CA1 synapse.
Academic Article	Sodium channel Scn1b null mice exhibit prolonged QT and RR intervals.
Academic Article	Rai1 deficiency in mice causes learning impairment and motor dysfunction, whereas Rai1 heterozygous mice display minimal behavioral phenotypes.
Academic Article	Synchronous hippocampal bursting reveals network excitability defects in an epilepsy gene mutation.
Academic Article	Sodium channel beta1 regulatory subunit deficiency reduces pancreatic islet glucose-stimulated insulin and glucagon secretion.
Academic Article	Expanded alternative splice isoform profiling of the mouse Cav3.1/alpha1G T-type calcium channel.
Academic Article	A triplet repeat expansion genetic mouse model of infantile spasms syndrome, Arx(GCG)10+7, with interneuronopathy, spasms in infancy, persistent seizures, and adult cognitive and behavioral impairment.
Academic Article	Stargazer: a new neurological mutant on chromosome 15 in the mouse with prolonged cortical seizures.
Academic Article	Altered hippocampal network excitability in the hypernoradrenergic mutant mouse tottering.
Academic Article	Arrhythmia in heart and brain: KCNQ1 mutations link epilepsy and sudden unexplained death.
Academic Article	Altered ultrasonic vocalization and impaired learning and memory in Angelman syndrome mouse model with a large maternal deletion from Ube3a to Gabrb3.
Academic Article	Kv1.1 potassium channel deficiency reveals brain-driven cardiac dysfunction as a candidate mechanism for sudden unexplained death in epilepsy.
Academic Article	Dysfunction in GABA signalling mediates autism-like stereotypies and Rett syndrome phenotypes.
Academic Article	A perfect storm: Converging paths of epilepsy and Alzheimer's dementia intersect in the hippocampal formation.
Academic Article	Amyloid-?/Fyn-induced synaptic, network, and cognitive impairments depend on tau levels in multiple mouse models of Alzheimer's disease.
Academic Article	Delayed postnatal loss of P/Q-type calcium channels recapitulates the absence epilepsy, dyskinesia, and ataxia phenotypes of genomic Cacna1a mutations.
Academic Article	MeCP2 is critical within HoxB1-derived tissues of mice for normal lifespan.
Academic Article	Suppression of PKR promotes network excitability and enhanced cognition by interferon-?-mediated disinhibition.
Academic Article	Tau loss attenuates neuronal network hyperexcitability in mouse and Drosophila genetic models of epilepsy.
Academic Article	NOVA-dependent regulation of cryptic NMD exons controls synaptic protein levels after seizure.
Academic Article	Postnatal loss of P/Q-type channels confined to rhombic-lip-derived neurons alters synaptic transmission at the parallel fiber to purkinje cell synapse and replicates genomic Cacna1a mutation phenotype of ataxia and seizures in mice.
Academic Article	Persistent hypersynchronization of neocortical neurons in the mocha mutant of mouse.
Academic Article	Analysis of inherited epilepsy using single locus mutations in mice.
Academic Article	Inherited epilepsy: spike-wave and focal motor seizures in the mutant mouse tottering.
Academic Article	Isolating single genes of the inherited epilepsies.
Academic Article	A single gene error of noradrenergic axon growth synchronizes central neurones.
Academic Article	Mutant mouse tottering: selective increase of locus ceruleus axons in a defined single-locus mutation.
Academic Article	Single locus mutations in mice expressing generalized spike-wave absence epilepsies.
Academic Article	Developmental analysis of hippocampal mossy fiber outgrowth in a mutant mouse with inherited spike-wave seizures.
Academic Article	Selective failure of brain-derived neurotrophic factor mRNA expression in the cerebellum of stargazer, a mutant mouse with ataxia.
Academic Article	Mutation of the Ca2+ channel beta subunit gene Cchb4 is associated with ataxia and seizures in the lethargic (lh) mouse.
Academic Article	Epilepsy in mice deficient in the 65-kDa isoform of glutamic acid decarboxylase.
Academic Article	Nonobligate role of early or sustained expression of immediate-early gene proteins c-fos, c-jun, and Zif/268 in hippocampal mossy fiber sprouting.
Academic Article	Single gene defects in mice: the role of voltage-dependent calcium channels in absence models.
Academic Article	Impaired neurotransmitter release and elevated threshold for cortical spreading depression in mice with mutations in the alpha1A subunit of P/Q type calcium channels.
Academic Article	Large expansion of the ATTCT pentanucleotide repeat in spinocerebellar ataxia type 10.
Academic Article	Modeling human epilepsies in mice.
Academic Article	Mutations in high-voltage-activated calcium channel genes stimulate low-voltage-activated currents in mouse thalamic relay neurons.
Academic Article	Genetic and phenotypic analysis of the mouse mutant mh2J, an Ap3d allele caused by IAP element insertion.
Academic Article	Modeling del(17)(p11.2p11.2) and dup(17)(p11.2p11.2) contiguous gene syndromes by chromosome engineering in mice: phenotypic consequences of gene dosage imbalance.
Academic Article	Exploring new gene discoveries in idiopathic generalized epilepsy.
Academic Article	Cathepsin B but not cathepsins L or S contributes to the pathogenesis of Unverricht-Lundborg progressive myoclonus epilepsy (EPM1).
Academic Article	Apoptosis caused by cathepsins does not require Bid signaling in an in vivo model of progressive myoclonus epilepsy (EPM1).
Academic Article	Sodium channel density in hypomyelinated brain increased by myelin basic protein gene deletion.
Academic Article	Expression of apoptosis inhibitor protein Mcl1 linked to neuroprotection in CNS neurons.
Academic Article	Mutational analysis of spike-wave epilepsy phenotypes.
Academic Article	Persistent hypersynchronization of neocortical neurons in the mocha mutant of mouse.
Academic Article	Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganization.
Academic Article	Genetic enhancement of thalamocortical network activity by elevating alpha 1g-mediated low-voltage-activated calcium current induces pure absence epilepsy.
Academic Article	Deletion of the potassium channel Kv12.2 causes hippocampal hyperexcitability and epilepsy.
Academic Article	Neuronal Elav-like (Hu) proteins regulate RNA splicing and abundance to control glutamate levels and neuronal excitability.
Academic Article	Novel brain expression of ClC-1 chloride channels and enrichment of CLCN1 variants in epilepsy.
Concept	Mice, Inbred CBA
Concept	Mice, Knockout
Concept	Mice, Inbred NOD
Concept	Mice, Inbred BALB C
Concept	Mice, Inbred C3H
Concept	Mice, Inbred Strains
Concept	Mice
Concept	Mice, Neurologic Mutants
Concept	Mice, Mutant Strains
Concept	Mice, Inbred C57BL
Concept	Mice, Transgenic
Academic Article	Paradoxical proepileptic response to NMDA receptor blockade linked to cortical interneuron defect in stargazer mice.
Academic Article	Neonatal estradiol stimulation prevents epilepsy in Arx model of X-linked infantile spasms syndrome.
Academic Article	Genetic suppression of transgenic APP rescues Hypersynchronous network activity in a mouse model of Alzeimer's disease.
Academic Article	Bexarotene reduces network excitability in models of Alzheimer's disease and epilepsy.
Academic Article	Reduced cognition in Syngap1 mutants is caused by isolated damage within developing forebrain excitatory neurons.
Academic Article	Hyper-SUMOylation of the Kv7 potassium channel diminishes the M-current leading to seizures and sudden death.
Academic Article	Compromised maturation of GABAergic inhibition underlies abnormal network activity in the hippocampus of epileptic Ca2+ channel mutant mice, tottering.
Academic Article	Spreading depolarization in the brainstem mediates sudden cardiorespiratory arrest in mouse SUDEP models.
Academic Article	Expression and function of Kv1.1 potassium channels in human atria from patients with atrial fibrillation.
Academic Article	Selective Loss of Presynaptic Potassium Channel Clusters at the Cerebellar Basket Cell Terminal Pinceau in Adam11 Mutants Reveals Their Role in Ephaptic Control of Purkinje Cell Firing.
Academic Article	Shift in interictal relative gamma power as a novel biomarker for drug response in two mouse models of absence epilepsy.
Academic Article	Isolated P/Q Calcium Channel Deletion in Layer VI Corticothalamic Neurons Generates Absence Epilepsy.
Academic Article	Leaky RyR2 channels unleash a brainstem spreading depolarization mechanism of sudden cardiac death.
Academic Article	Ankyrin-G isoform imbalance and interneuronopathy link epilepsy and bipolar disorder.
Academic Article	Brain microvasculature defects and Glut1 deficiency syndrome averted by early repletion of the glucose transporter-1 protein.
Academic Article	Identification of diverse astrocyte populations and their malignant analogs.
Academic Article	aII Spectrin Forms a Periodic Cytoskeleton at the Axon Initial Segment and Is Required for Nervous System Function.
Academic Article	Persistent aberrant cortical phase-amplitude coupling following seizure treatment in absence epilepsy models.
Academic Article	Nav1.2 haplodeficiency in excitatory neurons causes absence-like seizures in mice.
Academic Article	Asynchronous suppression of visual cortex during absence seizures in stargazer mice.
Academic Article	Adrenergic agonist induces rhythmic firing in quiescent cardiac preganglionic neurons in nucleus ambiguous via activation of intrinsic membrane excitability.
Grant	SUDEP Research Alliance: Cardiac Gene and Circuit Mechanisms; Application 7 of 7
Grant	NEUROPHYSIOLOGY DATABASE OF INBRED MUTANT STRAINS
Grant	PREDICTIVE GENES, MECHANISMS, AND CLINICAL BIOMARKERS OF SUDEP
Grant	In vivo recruitment of neocortical neurons in stargazer absence seizures
Academic Article	Brainstem spreading depolarization: rapid descent into the shadow of SUDEP.
Concept	Mice, 129 Strain
Academic Article	Therapeutic inhibition of mTORC2 rescues the behavioral and neurophysiological abnormalities associated with Pten-deficiency.
Academic Article	Adult loss of Cacna1a in mice recapitulates childhood absence epilepsy by distinct thalamic bursting mechanisms.
Academic Article	PIK3CA variants selectively initiate brain hyperactivity during gliomagenesis.
Academic Article	Arx expansion mutation perturbs cortical development by augmenting apoptosis without activating innate immunity in a mouse model of X-linked infantile spasms syndrome.
Academic Article	Pathogenesis of peritumoral hyperexcitability in an immunocompetent CRISPR-based glioblastoma model.
Academic Article	? spectrin-dependent and domain specific mechanisms for Na+ channel clustering.
Grant	Development of aberrant cortical interneuron circuitry in genetic mouse models of absence epilepsy
Academic Article	Early 17?-estradiol treatment reduces seizures but not abnormal behaviour in mice with expanded polyalanine tracts in the Aristaless related homeobox gene (ARX).
Academic Article	Kcnq2/Kv7.2 controls the threshold and bi-hemispheric symmetry of cortical spreading depolarization.
Academic Article	Loss of functional System x-c uncouples aberrant postnatal neurogenesis from epileptogenesis in the hippocampus of Kcna1-KO mice.
Academic Article	Emx1-Cre Is Expressed in Peripheral Autonomic Ganglia That Regulate Central Cardiorespiratory Functions.
Academic Article	Kctd7 deficiency induces myoclonic seizures associated with Purkinje cell death and microvascular defects.
Academic Article	Glioma epileptiform activity and progression are driven by IGSF3-mediated potassium dysregulation.
Academic Article	WWOX P47T partial loss-of-function mutation induces epilepsy, progressive neuroinflammation, and cerebellar degeneration in mice phenocopying human SCAR12.
Academic Article	Remote neuronal activity drives glioma progression through SEMA4F.
Academic Article	A hyperthermic seizure unleashes a surge of spreading depolarizations in Scn1a-deficient mice.
Academic Article	Targeted suppression of mTORC2 reduces seizures across models of epilepsy.
Academic Article	Abnormal patterns of sleep and waking behaviors are accompanied by neocortical oscillation disturbances in an Ank3 mouse model of epilepsy-bipolar disorder comorbidity.

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