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https://www.readbyqxmd.com/read/30104343/a-transient-developmental-window-of-fast-spiking-interneuron-dysfunction-in-a-mouse-model-of-dravet-syndrome
#1
Morgana Favero, Nathaniel P Sotuyo, Emily Lopez, Jennifer A Kearney, Ethan M Goldberg
Dravet syndrome is a severe childhood-onset epilepsy largely due to heterozygous loss-of-function mutation of the gene SCN1A , which encodes the type 1 neuronal voltage gated sodium (Na+ ) channel α subunit Nav1.1. Prior studies in mouse models of Dravet syndrome ( Scn1a +/- mice) indicate that, in cerebral cortex, Nav1.1 is predominantly expressed in GABAergic interneurons, in particular in parvalbumin-positive fast-spiking basket cells (PVINs). This has led to a model of Dravet syndrome pathogenesis in which Nav1...
August 13, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/30076230/selective-na-v-1-1-activation-rescues-dravet-syndrome-mice-from-seizures-and-premature-death
#2
Kay L Richards, Carol J Milligan, Robert J Richardson, Nikola Jancovski, Morten Grunnet, Laura H Jacobson, Eivind A B Undheim, Mehdi Mobli, Chun Yuen Chow, Volker Herzig, Agota Csoti, Gyorgy Panyi, Christopher A Reid, Glenn F King, Steven Petrou
Dravet syndrome is a catastrophic, pharmacoresistant epileptic encephalopathy. Disease onset occurs in the first year of life, followed by developmental delay with cognitive and behavioral dysfunction and substantially elevated risk of premature death. The majority of affected individuals harbor a loss-of-function mutation in one allele of SCN1A , which encodes the voltage-gated sodium channel NaV 1.1. Brain NaV 1.1 is primarily localized to fast-spiking inhibitory interneurons; thus the mechanism of epileptogenesis in Dravet syndrome is hypothesized to be reduced inhibitory neurotransmission leading to brain hyperexcitability...
August 3, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/30048010/remodeled-cortical-inhibition-prevents-motor-seizures-in-generalized-epilepsy
#3
Xiao Jiang, Alexis Lupien-Meilleur, Sabrina Tazerart, Mathieu Lachance, Elena Samarova, Roberto Araya, Jean-Claude Lacaille, Elsa Rossignol
OBJECTIVE: Deletions of CACNA1A, encoding the α1 subunit of CaV 2.1 channels, cause epilepsy with ataxia in humans. While the deletion of Cacna1a in GABAergic interneurons (INs) derived from the medial ganglionic eminence (MGE) impairs cortical inhibition and causes generalized seizures in Nkx2.1Cre ;Cacna1ac/c mice, the targeted deletion of Cacna1a in somatostatin-expressing INs (SOM-INs), a subset of MGE-derived INs, does not result in seizures, indicating a crucial role of parvalbumin-expressing (PV) INs...
July 26, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/30021052/dissecting-the-neuronal-vulnerability-underpinning-alpers-syndrome-a-clinical-and-neuropathological-study
#4
Hannah Hayhurst, Maria-Eleni Anagnostou, Helen J Bogle, John P Grady, Robert W Taylor, Laurence A Bindoff, Robert McFarland, Doug M Turnbull, Nichola Z Lax
Alpers' syndrome is an early-onset neurodegenerative disorder often caused by biallelic pathogenic variants in the gene encoding the catalytic subunit of polymerase-gamma (POLG) which is essential for mitochondrial DNA (mtDNA) replication. Alpers' syndrome is characterised by intractable epilepsy, developmental regression and liver failure which typically affects children aged 6 months - 3 years. Although later onset variants are now recognised, they differ in that they are primarily an epileptic encephalopathy with ataxia...
July 18, 2018: Brain Pathology
https://www.readbyqxmd.com/read/30013074/gabrb2-knockout-mice-displayed-schizophrenia-like-and-comorbid-phenotypes-with-interneuron-astrocyte-microglia-dysregulation
#5
Rigil K Yeung, Zheng-Hua Xiang, Shui-Ying Tsang, Rui Li, Timothy Y C Ho, Qi Li, Chok-King Hui, Pak-Chung Sham, Ming-Qi Qiao, Hong Xue
Intronic polymorphisms of the GABAA receptor β2 subunit gene (GABRB2) under adaptive evolution were associated with schizophrenia and reduced expression, especially of the long isoform which differs in electrophysiological properties from the short isoform. The present study was directed to examining the gene dosage effects of Gabrb2 in knockout mice of both heterozygous (HT) and homozygous (KO) genotypes with respect to possible schizophrenia-like and comorbid phenotypes. The KO mice, and HT mice to a lesser extent, were found to display prepulse inhibition (PPI) deficit, locomotor hyperactivity, stereotypy, sociability impairments, spatial-working and spatial-reference memory deficits, reduced depression and anxiety, and accelerated pentylenetetrazol (PTZ)-induced seizure...
July 17, 2018: Translational Psychiatry
https://www.readbyqxmd.com/read/29997478/cortical-gabaergic-interneuron-progenitor-transplantation-as-a-novel-therapy-for-intractable-epilepsy
#6
REVIEW
Qian Zhu, Janice R Naegele, Sangmi Chung
Epilepsy is a severe neurological disease affecting more than 70 million people worldwide that is characterized by unpredictable and abnormal electrical discharges resulting in recurrent seizures. Although antiepileptic drugs (AEDs) are the mainstay of epilepsy treatment for seizure control, about one third of patients with epilepsy suffer from intractable seizures that are unresponsive to AEDs. Furthermore, the patients that respond to AEDs typically experience adverse systemic side effects, underscoring the urgent need to develop new therapies that target epileptic foci rather than more systemic interventions...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29988380/kainate-receptors-role-in-epilepsy
#7
REVIEW
Rafael Falcón-Moya, Talvinder S Sihra, Antonio Rodríguez-Moreno
Kainate (KA) is a potent neurotoxin that has been widely used experimentally to induce acute brain seizures and, after repetitive treatments, as a chronic model of temporal lobe epilepsy (TLE), with similar features to those observed in human patients with TLE. However, whether KA activates KA receptors (KARs) as an agonist to mediate the induction of acute seizures and/or the chronic phase of epilepsy, or whether epileptogenic effects of the neurotoxin are indirect and/or mediated by other types of receptors, has yet to be satisfactorily elucidated...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29951184/role-of-mir-146a-in-neural-stem-cell-differentiation-and-neural-lineage-determination-relevance-for-neurodevelopmental-disorders
#8
Lam Son Nguyen, Julien Fregeac, Christine Bole-Feysot, Nicolas Cagnard, Anand Iyer, Jasper Anink, Eleonora Aronica, Olivier Alibeu, Patrick Nitschke, Laurence Colleaux
Background: MicroRNAs (miRNAs) are small, non-coding RNAs that regulate gene expression at the post-transcriptional level. miRNAs have emerged as important modulators of brain development and neuronal function and are implicated in several neurological diseases. Previous studies found miR-146a upregulation is the most common miRNA deregulation event in neurodevelopmental disorders such as autism spectrum disorder (ASD), epilepsy, and intellectual disability (ID). Yet, how miR-146a upregulation affects the developing fetal brain remains unclear...
2018: Molecular Autism
https://www.readbyqxmd.com/read/29947131/genome-wide-association-analysis-links-multiple-psychiatric-liability-genes-to-oscillatory-brain-activity
#9
Dirk J A Smit, Margaret J Wright, Jacquelyn L Meyers, Nicholas G Martin, Yvonne Y W Ho, Stephen M Malone, Jian Zhang, Scott J Burwell, David B Chorlian, Eco J C de Geus, Damiaan Denys, Narelle K Hansell, Jouke-Jan Hottenga, Matt McGue, Catharina E M van Beijsterveldt, Neda Jahanshad, Paul M Thompson, Christopher D Whelan, Sarah E Medland, Bernice Porjesz, William G Lacono, Dorret I Boomsma
Oscillatory activity is crucial for information processing in the brain, and has a long history as a biomarker for psychopathology. Variation in oscillatory activity is highly heritable, but current understanding of specific genetic influences remains limited. We performed the largest genome-wide association study to date of oscillatory power during eyes-closed resting electroencephalogram (EEG) across a range of frequencies (delta 1-3.75 Hz, theta 4-7.75 Hz, alpha 8-12.75 Hz, and beta 13-30 Hz) in 8,425 subjects...
June 26, 2018: Human Brain Mapping
https://www.readbyqxmd.com/read/29894724/pharmacological-rescue-of-mutated-k-v-3-1-ion-channel-linked-to-progressive-myoclonus-epilepsies
#10
Anders Sonne Munch, Arnela Saljic, Kim Boddum, Morten Grunnet, Charlotte Hougaard, Thomas Jespersen
Progressive myoclonus epilepsies (PMEs) constitute a cluster of inherent, genetically diverse, rare seizure disorders characterized by ataxia, tonic-clonic seizures, and action myoclonus. Recently, a mutation in the KCNC1 gene (Arg320His) was described in a group of PME patients. The KCNC1 gene encodes the Kv 3.1 potassium ion channel responsible for the rapid repolarization of the membrane potential following action potential firing in fast spiking GABAergic interneurons (FSI), thereby enabling high firing frequency...
August 15, 2018: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29889309/mechanisms-of-increased-hippocampal-excitability-in-the-mashl-mouse-model-of-na-k-atpase-dysfunction
#11
Arsen S Hunanyan, Ashley R Helseth, Elie Abdelnour, Bassil Kherallah, Monisha Sachdev, Leeyup Chung, Melanie Masoud, Jordan Richardson, Qiang Li, J Victor Nadler, Scott D Moore, Mohamad A Mikati
OBJECTIVE: Na+ /K+ -ATPase dysfunction, primary (mutation) or secondary (energy crisis, neurodegenerative disease) increases neuronal excitability in the brain. To evaluate the mechanisms underlying such increased excitability we studied mice carrying the D801N mutation, the most common mutation causing human disease, specifically alternating hemiplegia of childhood (AHC) including epilepsy. Because the gene is expressed in all neurons, particularly γ-aminobutyric acid (GABA)ergic interneurons, we hypothesized that the pathophysiology would involve both pyramidal cells and interneurons and that fast-spiking interneurons, which have increased firing rates, would be most vulnerable...
July 2018: Epilepsia
https://www.readbyqxmd.com/read/29766029/seizure-suppressant-and-neuroprotective-effects-of-encapsulated-bdnf-producing-cells-in-a-rat-model-of-temporal-lobe-epilepsy
#12
Chiara Falcicchia, Giovanna Paolone, Dwaine F Emerich, Francesca Lovisari, William J Bell, Tracie Fradet, Lars U Wahlberg, Michele Simonato
Brain-derived neurotrophic factor (BDNF) may represent a therapeutic for chronic epilepsy, but evaluating its potential is complicated by difficulties in its delivery to the brain. Here, we describe the effects on epileptic seizures of encapsulated cell biodelivery (ECB) devices filled with genetically modified human cells engineered to release BDNF. These devices, implanted into the hippocampus of pilocarpine-treated rats, highly decreased the frequency of spontaneous seizures by more than 80%. These benefits were associated with improved cognitive performance, as epileptic rats treated with BDNF performed significantly better on a novel object recognition test...
June 15, 2018: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/29659809/a-new-mouse-model-of-arx-dup24-recapitulates-the-patients-behavioral-and-fine-motor-alterations
#13
Aline Dubos, Hamid Meziane, Giovanni Iacono, Aurore Curie, Fabrice Riet, Christelle Martin, Nadège Loaëc, Marie-Christine Birling, Mohammed Selloum, Elisabeth Normand, Guillaume Pavlovic, Tania Sorg, Henk G Stunnenberg, Jamel Chelly, Yann Humeau, Gaëlle Friocourt, Yann Hérault
The aristaless-related homeobox (ARX) transcription factor is involved in the development of GABAergic and cholinergic neurons in the forebrain. ARX mutations have been associated with a wide spectrum of neurodevelopmental disorders in humans, among which the most frequent, a 24 bp duplication in the polyalanine tract 2 (c.428_451dup24), gives rise to intellectual disability, fine motor defects with or without epilepsy. To understand the functional consequences of this mutation, we generated a partially humanized mouse model carrying the c...
June 15, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29621484/rbfox1-regulates-synaptic-transmission-through-the-inhibitory-neuron-specific-vsnare-vamp1
#14
Celine K Vuong, Weizheng Wei, Ji-Ann Lee, Chia-Ho Lin, Andrey Damianov, Luis de la Torre-Ubieta, Reem Halabi, Klara Olofsdotter Otis, Kelsey C Martin, Thomas J O'Dell, Douglas L Black
Dysfunction of the neuronal RNA binding protein RBFOX1 has been linked to epilepsy and autism spectrum disorders. Rbfox1 loss in mice leads to neuronal hyper-excitability and seizures, but the physiological basis for this is unknown. We identify the vSNARE protein Vamp1 as a major Rbfox1 target. Vamp1 is strongly downregulated in Rbfox1 Nes-cKO mice due to loss of 3' UTR binding by RBFOX1. Cytoplasmic Rbfox1 stimulates Vamp1 expression in part by blocking microRNA-9. We find that Vamp1 is specifically expressed in inhibitory neurons, and that both Vamp1 knockdown and Rbfox1 loss lead to decreased inhibitory synaptic transmission and E/I imbalance...
April 4, 2018: Neuron
https://www.readbyqxmd.com/read/29574880/cannabidiol-exerts-antiepileptic-effects-by-restoring-hippocampal-interneuron-functions-in-a-temporal-lobe-epilepsy-model
#15
Archie A Khan, Tawfeeq Shekh-Ahmad, Ayatakin Khalil, Matthew C Walker, Afia B Ali
BACKGROUND AND PURPOSE: A non-psychoactive phytocannabinoid, cannabidiol (CBD), shows promising results as an effective potential antiepileptic drug in some forms of refractory epilepsy. To elucidate the mechanisms by which CBD exerts its anti-seizure effects, we investigated its effects at synaptic connections and on the intrinsic membrane properties of hippocampal CA1 pyramidal cells and two major inhibitory interneurons: fast spiking, parvalbumin (PV)-expressing and adapting, cholecystokinin (CCK)-expressing interneurons...
June 2018: British Journal of Pharmacology
https://www.readbyqxmd.com/read/29408225/partial-trkb-receptor-activation-suppresses-cortical-epileptogenesis-through-actions-on-parvalbumin-interneurons
#16
Feng Gu, Isabel Parada, Tao Yang, Frank M Longo, David A Prince
Post-traumatic epilepsy is one of the most common and difficult to treat forms of acquired epilepsy worldwide. Currently, there is no effective way to prevent post-traumatic epileptogenesis. It is known that abnormalities of interneurons, particularly parvalbumin-containing interneurons, play a critical role in epileptogenesis following traumatic brain injury. Thus, enhancing the function of existing parvalbumin interneurons might provide a logical therapeutic approach to prevention of post-traumatic epilepsy...
May 2018: Neurobiology of Disease
https://www.readbyqxmd.com/read/29377906/enhanced-susceptibility-to-stress-and-seizures-in-gad65-deficient-mice
#17
Jin Qi, Minjung Kim, Russell Sanchez, Saba M Ziaee, Jhumku D Kohtz, Sookyong Koh
Reduced gamma-aminobutyric acid (GABA) inhibition has been implicated in both anxiety and epilepsy. GAD65-/- (NOD/LtJ) mice have significantly decreased basal GABA levels in the brain and a lowered threshold for seizure generation. One fifth of GAD65 -/- mice experienced stress-induced seizures upon exposure to an open field at 4 weeks of age. In each successive week until 8 weeks of age, the latency to seizures decreased with prior seizure experience. 100% of GAD65-/- mice exhibited stress-induced seizures by the end of 8 weeks...
2018: PloS One
https://www.readbyqxmd.com/read/29302214/antidepressant-drug-paroxetine-blocks-the-open-pore-of-kv3-1-potassium-channel
#18
Hyang Mi Lee, Ok Hee Chai, Sang June Hahn, Bok Hee Choi
In patients with epilepsy, depression is a common comorbidity but difficult to be treated because many antidepressants cause pro-convulsive effects. Thus, it is important to identify the risk of seizures associated with antidepressants. To determine whether paroxetine, a very potent selective serotonin reuptake inhibitor (SSRI), interacts with ion channels that modulate neuronal excitability, we examined the effects of paroxetine on Kv3.1 potassium channels, which contribute to highfrequency firing of interneurons, using the whole-cell patch-clamp technique...
January 2018: Korean Journal of Physiology & Pharmacology
https://www.readbyqxmd.com/read/29286389/differentiation-of-mouse-embryonic-stem-cells-into-cortical-interneuron-precursors
#19
David J Tischfield, Stewart A Anderson
GABAergic cortical interneurons are a heterogeneous population of cells that play critical roles in regulating the output of excitatory pyramidal neurons as well as synchronizing the outputs of pyramidal neuron ensembles. Deficits in interneuron function have been implicated in a variety of neuropsychiatric disorders, including schizophrenia, autism, and epilepsy. The derivation of cortical interneurons from embryonic stem cells not only allows for the study of their development and function, but provides insight into the molecular mechanisms underlying the pathogenesis of cortical interneuron-related disorders...
December 3, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29229852/regulatory-networks-specifying-cortical-interneurons-from-human-embryonic-stem-cells-reveal-roles-for-chd2-in-interneuron-development
#20
Kesavan Meganathan, Emily M A Lewis, Paul Gontarz, Shaopeng Liu, Edouard G Stanley, Andrew G Elefanty, James E Huettner, Bo Zhang, Kristen L Kroll
Cortical interneurons (cINs) modulate excitatory neuronal activity by providing local inhibition. During fetal development, several cIN subtypes derive from the medial ganglionic eminence (MGE), a transient ventral telencephalic structure. While altered cIN development contributes to neurodevelopmental disorders, the inaccessibility of human fetal brain tissue during development has hampered efforts to define molecular networks controlling this process. Here, we modified protocols for directed differentiation of human embryonic stem cells, obtaining efficient, accelerated production of MGE-like progenitors and MGE-derived cIN subtypes with the expected electrophysiological properties...
December 26, 2017: Proceedings of the National Academy of Sciences of the United States of America
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