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epilepsy mouse model

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https://www.readbyqxmd.com/read/29466841/seizures-and-disturbed-brain-potassium-dynamics-in-the-leukodystrophy-mlc
#1
Mohit Dubey, Eelke Brouwers, Eline M C Hamilton, Oliver Stiedl, Marianna Bugiani, Henner Koch, Maarten H P Kole, Ursula Boschert, Robert C Wykes, Huibert D Mansvelder, Marjo S van der Knaap, Rogier Min
OBJECTIVE: Loss of function of the astrocyte-specific protein MLC1 leads to the childhood onset leukodystrophy "megalencephalic leukoencephalopathy with subcortical cysts" (MLC). Studies on isolated cells show a role for MLC1 in astrocyte volume regulation and suggest that disturbed brain ion and water homeostasis is central to the disease. Excitability of neuronal networks is particularly sensitive to ion and water homeostasis. In line with this, reports of seizures and epilepsy in MLC patients exist...
February 21, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29449490/dentate-gyrus-mossy-cells-control-spontaneous-convulsive-seizures-and-spatial-memory
#2
Anh D Bui, Theresa M Nguyen, Charles Limouse, Hannah K Kim, Gergely G Szabo, Sylwia Felong, Mattia Maroso, Ivan Soltesz
Temporal lobe epilepsy (TLE) is characterized by debilitating, recurring seizures and an increased risk for cognitive deficits. Mossy cells (MCs) are key neurons in the hippocampal excitatory circuit, and the partial loss of MCs is a major hallmark of TLE. We investigated how MCs contribute to spontaneous ictal activity and to spatial contextual memory in a mouse model of TLE with hippocampal sclerosis, using a combination of optogenetic, electrophysiological, and behavioral approaches. In chronically epileptic mice, real-time optogenetic modulation of MCs during spontaneous hippocampal seizures controlled the progression of activity from an electrographic to convulsive seizure...
February 16, 2018: Science
https://www.readbyqxmd.com/read/29443088/simultaneous-video-eeg-ecg-monitoring-to-identify-neurocardiac-dysfunction-in-mouse-models-of-epilepsy
#3
Vikas Mishra, Nicole M Gautier, Edward Glasscock
In epilepsy, seizures can evoke cardiac rhythm disturbances such as heart rate changes, conduction blocks, asystoles, and arrhythmias, which can potentially increase risk of sudden unexpected death in epilepsy (SUDEP). Electroencephalography (EEG) and electrocardiography (ECG) are widely used clinical diagnostic tools to monitor for abnormal brain and cardiac rhythms in patients. Here, a technique to simultaneously record video, EEG, and ECG in mice to measure behavior, brain, and cardiac activities, respectively, is described...
January 29, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29425964/alterations-of-l-type-voltage-dependent-calcium-channel-alpha-1-subunit-in-the-hippocampal-ca3-region-during-and-after-pilocarpine-induced-epilepsy
#4
Jie Hua Xu, Hui Wang, Wen Zhang, Feng Ru Tang
Voltage-dependent calcium channels (VDCC) have been shown to regulate neuronal excitability and their antagonists have been used clinically for the control of seizures. While functional studies of VDCC in epileptogenesis in the CA1 area of hippocampus or the dentate gyrus have been done, few studies were carried out in the CA3 area. Given the bursting characteristics of the CA3 neurons, we speculated that VDCC in the CA3 area might play an important role in the epileptogenesis. In the present study in the mouse pilocarpine model of temporal lobe epilepsy, we investigated the alterations of alpha 1 subunits of L-type VDCC in the CA3 area of the hippocampus at different stages of epileptogenesis, i...
February 7, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29414525/electrographic-and-pharmacological-characterization-of-a-progressive-epilepsy-phenotype-in-female-mecp2-deficient-mice
#5
Robert G Wither, Sinisa Colic, Berj L Bardakjian, O Carter Snead, Liang Zhang, James H Eubanks
Rett Syndrome is a neurodevelopmental disorder caused primarily by mutations in the gene encoding Methyl-CpG-binding protein 2 (MECP2). Spontaneous epileptiform activity is a common co-morbidity present in Rett syndrome, and hyper-excitable neural networks are present in MeCP2-deficient mouse models of Rett syndrome. In this study we conducted a longitudinal assessment of spontaneous cortical electrographic discharges in female MeCP2-deficient mice and defined the pharmacological responsiveness of these discharges to anti-convulsant drugs...
January 26, 2018: Epilepsy Research
https://www.readbyqxmd.com/read/29408991/astrocytes-new-players-in-progressive-myoclonus-epilepsy-of-lafora-type
#6
Carla Rubio-Villena, Rosa Viana, Jose Bonet, Maria Adelaida Garcia-Gimeno, Marta Casado, Miguel Heredia, Pascual Sanz
Lafora disease (LD) is a fatal form of progressive myoclonus epilepsy characterized by the accumulation of insoluble poorly branched glycogen-like inclusions named Lafora bodies (LBs) in the brain and peripheral tissues. In the brain, since its first discovery in 1911, it was assumed that these glycogen inclusions were only present in affected neurons. Mouse models of LD have been obtained recently, and we and others have been able to report the accumulation of glycogen inclusions in the brain of LD animals, what recapitulates the hallmark of the disease...
February 1, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29399113/geniposide-attenuates-epilepsy-symptoms-in-a-mouse-model-through-the-pi3k-akt-gsk-3%C3%AE-signaling-pathway
#7
Hongtao Wei, Guanghui Duan, Jianxun He, Qinglong Meng, Yuxian Liu, Wanqiang Chen, Yongpeng Meng
Previous reports on the pharmacological actions of geniposide have indicated that it has anti-asthmatic, anti-inflammatory and analgesic effects in the liver and gallbladder, and therapeutic effects in neurological, cardiovascular and cerebrovascular diseases. The results of the current study demonstrate that geniposide attenuates epilepsy in a mouse model through the phosphoinositide 3-kinase (PI3K)/protein kinase B (Akt)/glycogen synthase kinase-3β (GSK-3β) signaling pathway. A mouse model of epilepsy was induced by maximal electric shock (50 mA, 50 Hz, 1 sec)...
January 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29395074/otud7a-regulates-neurodevelopmental-phenotypes-in-the-15q13-3-microdeletion-syndrome
#8
Mohammed Uddin, Brianna K Unda, Vickie Kwan, Nicholas T Holzapfel, Sean H White, Leon Chalil, Marc Woodbury-Smith, Karen S Ho, Erin Harward, Nadeem Murtaza, Biren Dave, Giovanna Pellecchia, Lia D'Abate, Thomas Nalpathamkalam, Sylvia Lamoureux, John Wei, Marsha Speevak, James Stavropoulos, Kristin J Hope, Brad W Doble, Jacob Nielsen, E Robert Wassman, Stephen W Scherer, Karun K Singh
Copy-number variations (CNVs) are strong risk factors for neurodevelopmental and psychiatric disorders. The 15q13.3 microdeletion syndrome region contains up to ten genes and is associated with numerous conditions, including autism spectrum disorder (ASD), epilepsy, schizophrenia, and intellectual disability; however, the mechanisms underlying the pathogenesis of 15q13.3 microdeletion syndrome remain unknown. We combined whole-genome sequencing, human brain gene expression (proteome and transcriptome), and a mouse model with a syntenic heterozygous deletion (Df(h15q13)/+ mice) and determined that the microdeletion results in abnormal development of cortical dendritic spines and dendrite outgrowth...
February 1, 2018: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29384695/mouse-models-of-slc4-linked-disorders-of-hco3-transporter-dysfunction
#9
Mark D Parker
The SLC4 family Cl--HCO3- exchangers (AE1, AE2, and AE3) and Na+/HCO3- cotransporters (NBCe1, NBCe2, NBCn1, and NBCn2) contribute to a variety of vital physiological processes including pH regulation and epithelial fluid secretion. Accordingly, their dysfunction can have devastating effects. Disorders such as epilepsy, hemolytic anemia, glaucoma, hearing loss, osteopetrosis and renal tubular acidosis are all genetically linked to SLC4-family gene loci. This review summarizes how studies of Slc4-modified mice have enhanced our understanding of the etiology of SLC4-linked pathologies and the interpretation of genetic linkage studies...
January 31, 2018: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/29365285/ectopic-expression-of-miro-1-ameliorates-seizure-and-inhibits-hippocampal-neurodegeneration-in-a-mouse-pilocarpine-epilepsy-model
#10
Haifeng Zhang, Yajun Lian, Nanchang Xie, Yake Zheng
Epilepsy is a common disease of the central nervous system. This study aims to investigate the role of mitochondrial Rho (Miro) 1 in epilepsy using a mouse model of pilocarpine-induced status epilepticus (SE). Intraperitoneal injection of pilocarpine induced epileptic seizure in mice and significantly decreased Miro 1 expression in the hippocampus. Moreover, pilocarpine treatment increased the serum levels of heat shock protein 70 (HSP70) and S100 calcium binding protein B (S100B), and led to hippocampal neuronal injury and apoptosis...
January 24, 2018: Biochemistry and Cell Biology, Biochimie et Biologie Cellulaire
https://www.readbyqxmd.com/read/29364511/-18-f-ge180-positron-emission-tomographic-imaging-indicates-a-potential-double-hit-insult-in-the-intrahippocampal-kainate-mouse-model-of-temporal-lobe-epilepsy
#11
Mirjam Brackhan, Pablo Bascuñana, Tobias L Ross, Frank M Bengel, Jens P Bankstahl, Marion Bankstahl
OBJECTIVE: Accumulating evidence suggests that brain inflammation, elicited by epileptogenic insults, is involved in epilepsy development. Noninvasive nuclear imaging of brain inflammation in animal models of epileptogenesis represents a diagnostic in vivo approach with potential for direct translation into the clinic. Here, we investigated up-regulation of the translocator protein (TSPO) indicative of microglial activation by serial [18 F]GE180 positron emission tomographic (PET) imaging in a mouse model of temporal lobe epilepsy...
January 24, 2018: Epilepsia
https://www.readbyqxmd.com/read/29360985/temporal-requirement-of-dystroglycan-glycosylation-during-brain-development-and-rescue-of-severe-cortical-dysplasia-via-gene-delivery-in-the-fetal-stage
#12
Atsushi Sudo, Motoi Kanagawa, Mai Kondo, Chiyomi Ito, Kazuhiro Kobayashi, Mitsuharu Endo, Yasuhiro Minami, Atsu Aiba, Tatsushi Toda
Congenital muscular dystrophies (CMDs) are characterized by progressive weakness and degeneration of skeletal muscle. In several forms of CMD, abnormal glycosylation of α-dystroglycan (α-DG) results in conditions collectively known as dystroglycanopathies, which are associated with central nervous system involvement. We recently demonstrated that fukutin, the gene responsible for Fukuyama congenital muscular dystrophy, encodes the ribitol-phosphate transferase essential for dystroglycan function. Brain pathology in patients with dystroglycanopathy typically includes cobblestone lissencephaly, mental retardation, and refractory epilepsy; however, some patients exhibit average intelligence, with few or almost no structural defects...
January 19, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29360159/potent-and-selective-pharmacodynamic-synergy-between-the-metabotropic-glutamate-receptor-subtype-2-positive-allosteric-modulator-jnj-46356479-and-levetiracetam-in-the-mouse-6-hz-44-ma-model
#13
Cameron S Metcalf, Brian D Klein, Misty D Smith, Marc Ceusters, Hilde Lavreysen, Stefan Pype, Nancy Van Osselaer, Roy Twyman, H Steve White
OBJECTIVE: We previously demonstrated that positive allosteric modulators (PAMs) of metabotropic glutamate subtype 2 (mGlu2 ) receptors have potential synergistic interactions with the antiseizure drug levetiracetam (LEV). The present study utilizes isobolographic analysis to evaluate the combined administration of JNJ-46356479, a selective and potent mGlu2 PAM, with LEV as well as sodium valproate (VPA) and lamotrigine (LTG). METHODS: The anticonvulsant efficacy of JNJ-46356479 was evaluated in the 6-Hz model of psychomotor seizures in mice...
January 23, 2018: Epilepsia
https://www.readbyqxmd.com/read/29337673/evaluation-of-immunomodulatory-effects-of-lamotrigine-in-balb-c-mice
#14
Eman Y Abu-Rish, Shada Y Elhayek, Yehia S Mohamed, Islam Hamad, Yasser Bustanji
Modulation of the immune system has recently been shown to be involved in the pharmacological effects of old antiepileptic drugs and in the pathogenesis of epilepsy. Therefore, the most recent guidelines for immunotoxicological evaluation of drugs were consulted to investigate the immunomodulatory effects of lamotrigine, a newer antiepileptic drug, in BALB/c mice. These included the in vivo effects of lamotrigine on delayed-type hypersensitivity (DTH) response to sheep red blood cell (SRBC) antigens, hemagglutination titer assays and hematological changes...
December 20, 2017: Acta Pharmaceutica
https://www.readbyqxmd.com/read/29329017/amiodarone-a-multi-channel-blocker-enhances-anticonvulsive-effect-of-carbamazepine-in-the-mouse-maximal-electroshock-model
#15
Monika Banach, Monika Popławska, Kinga K Borowicz-Reutt
Cardiac arrhythmia may occur in the course of epilepsy. Simultaneous therapy of the two diseases might be complicated by drug interactions since antiarrhythmic and antiepileptic agents share some molecular targets. The aim of this study was to evaluate the influence of amiodarone, an antiarrhythmic drug working as a multi-channel blocker, on the protective activity of four classical antiepileptic drugs in the maximal electroshock test in mice. Amiodarone at doses up to 75 mg/kg did not affect the electroconvulsive threshold in mice...
January 5, 2018: Epilepsy Research
https://www.readbyqxmd.com/read/29317669/selective-targeting-of-scn8a-prevents-seizure-development-in-a-mouse-model-of-mesial-temporal-lobe-epilepsy
#16
Jennifer C Wong, Christopher D Makinson, Tyra Lamar, Qi Cheng, Jeffrey C Wingard, Ernest F Terwilliger, Andrew Escayg
We previously found that genetic mutants with reduced expression or activity of Scn8a are resistant to induced seizures and that co-segregation of a mutant Scn8a allele can increase survival and seizure resistance of Scn1a mutant mice. In contrast, Scn8a expression is increased in the hippocampus following status epilepticus and amygdala kindling. These findings point to Scn8a as a promising therapeutic target for epilepsy and raise the possibility that aberrant overexpression of Scn8a in limbic structures may contribute to some epilepsies, including temporal lobe epilepsy...
January 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29274432/a-mouse-model-of-depdc5-related-epilepsy-neuronal-loss-of-depdc5-causes-dysplastic-and-ectopic-neurons-increased-mtor-signaling-and-seizure-susceptibility
#17
Christopher J Yuskaitis, Brandon M Jones, Rachel L Wolfson, Chloe E Super, Sameer C Dhamne, Alexander Rotenberg, David M Sabatini, Mustafa Sahin, Annapurna Poduri
DEPDC5 is a newly identified epilepsy-related gene implicated in focal epilepsy, brain malformations, and Sudden Unexplained Death in Epilepsy (SUDEP). In vitro, DEPDC5 negatively regulates amino acid sensing by the mTOR complex 1 (mTORC1) pathway, but the role of DEPDC5 in neurodevelopment and epilepsy has not been described. No animal model of DEPDC5-related epilepsy has recapitulated the neurological phenotypes seen in patients, and germline knockout rodent models are embryonic lethal. Here, we establish a neuron-specific Depdc5 conditional knockout mouse by cre-recombination under the Synapsin1 promotor...
December 20, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/29274430/divergent-brain-changes-in-two-audiogenic-rat-strains-a-voxel-based-morphometry-and-diffusion-tensor-imaging-comparison-of-the-genetically-epilepsy-prone-rat-gepr-3-and-the-wistar-audiogenic-rat-war
#18
Yichien Lee, Olga C Rodriguez, Chris Albanese, Victor Rodrigues Santos, José Antônio Cortes de Oliveira, Ana Luiza Ferreira Donatti, Artur Fernandes, Norberto Garcia-Cairasco, Prosper N'Gouemo, Patrick A Forcelli
Acoustically evoked seizures (e.g., audiogenic seizures or AGS) are common in models of inherited epilepsy and occur in a variety of species including rat, mouse, and hamster. Two models that have been particularly well studied are the genetically epilepsy prone rat (GEPR-3) and the Wistar Audiogenic Rat (WAR) strains. Acute and repeated AGS, as well as comorbid conditions, displays a close phenotypic overlap in these models. Whether these similarities arise from convergent or divergent structural changes in the brain remains unknown...
December 20, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/29273763/interactions-between-ghrh-and-gabaars-in-the-brains-of-patients-with-epilepsy-and-in-animal-models-of-epilepsy
#19
Shirong Tang, Zhong Luo, Xiaowei Qiu, Yanke Zhang, Xi Lu, Hao Huang, Zhongxiang Xu, Zucai Xu
Growth hormone releasing hormone (GHRH) has recently been shown to increase the level of γ-aminobutyric acid (GABA) and activate GABA receptors (GABARs) in the cerebral cortex. GABA is an inhibitory neurotransmitter that can inhibit seizures. Does GHRH enhance the inhibitory effect of GABA to prevent epilepsy by increasing the GABA level and activating GABARs? In this study, patients with epilepsy and C57/BL6 mice with epilepsy induced by kainic acid (KA) or pentylenetetrazol (PTZ) served as the research subjects...
December 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29246093/targeting-the-mammalian-target-of-rapamycin-for-epileptic-encephalopathies-and-malformations-of-cortical-development
#20
Anna Jeong, Michael Wong
Malformations of cortical development represent a common cause of epileptic encephalopathies and drug-resistant epilepsy in children. As current treatments are often ineffective, new therapeutic targets are needed for epileptic encephalopathies associated with cortical malformations. The mechanistic/mammalian target of rapamycin (mTOR) pathway constitutes a signaling pathway that drives cellular and molecular mechanisms of epileptogenesis in a variety of focal cortical malformations. mTOR inhibitors prevent epilepsy and associated pathogenic mechanisms of epileptogenesis in mouse models of tuberous sclerosis complex and are currently in clinical trials for drug-resistant seizures in these patients...
January 2018: Journal of Child Neurology
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