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https://www.readbyqxmd.com/read/28752895/development-of-resurgent-and-persistent-sodium-currents-in-mesencephalic-trigeminal-neurons
#1
Akifumi Enomoto, Soju Seki, Susumu Tanaka, Kohji Ishihama, Tadashi Yamanishi, Mikihiko Kogo, Suguru Hamada
Sodium channels play multiple roles in the formation of neural membrane properties in mesencephalic trigeminal (Mes V) neurons and in other neural systems. Mes V neurons exhibit conditional robust high-frequency spike discharges. As previously reported, resurgent and persistent sodium currents (INaR and INaP , respectively) may carry small currents at subthreshold voltages that contribute to generation of spike firing. These currents play an important role in maintaining and allowing high-frequency spike discharge during a burst...
July 28, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28747464/lethal-digenic-mutations-in-the-k-channels-kir4-1-kcnj10-and-slack-kcnt1-associated-with-severe-disabling-seizures-and-neurodevelopmental-delay
#2
Sonia Majed Hasan, Ameera Balobaid, Alessandro Grottesi, Omar Dabbagh, Marta Cenciarini, Rifaat Rawashdeh, Afaf Al-Sagheir, Cecilia Bove, Lara Macchioni, Mauro Pessia, Mohammed Al-Owain, Maria Cristina D'Adamo
A 2-year-old boy presented profound developmental delay, failure to thrive, ataxia, hypotonia and tonic-clonic seizures that caused the death of the patient. Targeted and whole-exome sequencing revealed two heterozygous missense variants: a novel mutation in KCNJ10 gene that encodes for the inwardly-rectifying K(+) channel Kir4.1 and another previously characterized mutation in KCNT1 that encodes for the Na(+)-activated K(+) channel known as Slo2.2 or SLACK. The objectives of this study were to perform the clinical and genetic characterization of the proband and his family and to examine the functional consequence of the Kir4...
July 26, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28676574/aberrant-sodium-channel-currents-and-hyperexcitability-of-medial-entorhinal-cortex-neurons-in-a-mouse-model-of-scn8a-encephalopathy
#3
Matteo Ottolini, Bryan S Barker, Ronald P Gaykema, Miriam H Meisler, Manoj K Patel
SCN8A encephalopathy, or early infantile epileptic encephalopathy 13 (EIEE13), is caused predominantly by de novo gain-of-function mutations in the voltage-gated Na channel Nav1.6. Affected individuals suffer from refractory seizures, developmental delay, cognitive disability, and elevated risk of sudden unexpected death in epilepsy (SUDEP). A knock-in mouse model carrying the patient mutation p.Asn1768Asp (N1768D) reproduces many features of the disorder, including spontaneous seizures and SUDEP. We used the mouse model to examine the effects of the mutation on layer II stellate neurons of the medial entorhinal cortex (mEC), which transmit excitatory input to the hippocampus...
August 9, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28554310/ion-channel-and-neurotransmitter-modulators-as-electroceutical-approaches-to-the-control-of-cancer
#4
Jack Tuszynski, Tatiana M Tilli, Michael Levin
The activities of individual cells must be tightly coordinated in order to build and maintain complex 3-dimensional body structures during embryogenesis and regeneration. Thus, one way to view cancer is within systems biology as a network disorder affecting the ability of cells to properly interact with a morphodynamic field of instructive signals that keeps proliferation and migration orchestrated toward the anatomical needs of the host organism. One layer of this set of instructive microenvironmental cues is bioelectrical...
May 29, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28524267/synaptic-activity-suppresses-expression-of-neurogenic-differentiation-factor-2-in-an-nmda-receptor-dependent-manner
#5
Fading Chen, Benjamin J Hall
Neurogenic differentiation factor 2 (NeuroD2) is a highly expressed transcription factor in the developing central nervous system. In newborn neurons, NeuroD2-mediated gene expression promotes differentiation, maturation, and survival. In addition to these early, cell-intrinsic developmental processes, NeuroD2 in postmitotic neurons also regulates synapse growth and ion channel expression to control excitability. While NeuroD2 transactivation can be induced in an activity-dependent manner, little is known about how expression of NeuroD2 itself is regulated...
May 19, 2017: Synapse
https://www.readbyqxmd.com/read/28416002/biological-effects-of-exposure-to-static-electric-fields-in-humans-and-vertebrates-a-systematic-review
#6
REVIEW
Anne-Kathrin Petri, Kristina Schmiedchen, Dominik Stunder, Dagmar Dechent, Thomas Kraus, William H Bailey, Sarah Driessen
BACKGROUND: High-voltage direct current (HVDC) lines are the technology of choice for the transport of large amounts of energy over long distances. The operation of these lines produces static electric fields (EF), but the data reviewed in previous assessments were not sufficient to assess the need for any environmental limit. The aim of this systematic review was to update the current state of research and to evaluate biological effects of static EF. METHODS: Using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses) recommendations, we collected and evaluated experimental and epidemiological studies examining biological effects of exposure to static EF in humans (n = 8) and vertebrates (n = 40)...
April 17, 2017: Environmental Health: a Global Access Science Source
https://www.readbyqxmd.com/read/28255270/activated-clc-2-inhibits-p-akt-to-repress-myelination-in-gdm-newborn-rats
#7
Feixiang He, Yuchen Peng, Zhi Yang, Zilu Ge, Yanping Tian, Teng Ma, Hongli Li
This study aims to investigate the effect and mechanism of type 2 voltage-gated chloride channel (ClC-2) on myelin development of newborn rats' cerebral white matter with gestational diabetes mellitus (GDM). In this study, GDM model was induced in late pregnant rat model. The alteration of ClC-2 expression in various developmental stages of cerebral white matter with/without being exposed to high glucose was analyzed using RT-PCR, active oxygen detection, TUNEL staining, Western Blot as well as immuno-histochemical staining...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28235671/scn3a-deficiency-associated-with-increased-seizure-susceptibility
#8
Tyra Lamar, Carlos G Vanoye, Jeffrey Calhoun, Jennifer C Wong, Stacey B B Dutton, Benjamin S Jorge, Milen Velinov, Andrew Escayg, Jennifer A Kearney
Mutations in voltage-gated sodium channels expressed highly in the brain (SCN1A, SCN2A, SCN3A, and SCN8A) are responsible for an increasing number of epilepsy syndromes. In particular, mutations in the SCN3A gene, encoding the pore-forming Nav1.3 α subunit, have been identified in patients with focal epilepsy. Biophysical characterization of epilepsy-associated SCN3A variants suggests that both gain- and loss-of-function SCN3A mutations may lead to increased seizure susceptibility. In this report, we identified a novel SCN3A variant (L247P) by whole exome sequencing of a child with focal epilepsy, developmental delay, and autonomic nervous system dysfunction...
June 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28202726/two-tonoplast-mate-proteins-function-as-turgor-regulating-chloride-channels-in-arabidopsis
#9
Haiwen Zhang, Fu-Geng Zhao, Ren-Jie Tang, Yuexuan Yu, Jiali Song, Yuan Wang, Legong Li, Sheng Luan
The central vacuole in a plant cell occupies the majority of the cellular volume and plays a key role in turgor regulation. The vacuolar membrane (tonoplast) contains a large number of transporters that mediate fluxes of solutes and water, thereby adjusting cell turgor in response to developmental and environmental signals. We report that two tonoplast Detoxification efflux carrier (DTX)/Multidrug and Toxic Compound Extrusion (MATE) transporters, DTX33 and DTX35, function as chloride channels essential for turgor regulation in Arabidopsis Ectopic expression of each transporter in Nicotiana benthamiana mesophyll cells elicited a large voltage-dependent inward chloride current across the tonoplast, showing that DTX33 and DTX35 each constitute a functional channel...
March 7, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28193459/epileptiform-activity-and-behavioral-arrests-in-mice-overexpressing-the-calcium-channel-subunit-%C3%AE-2%C3%AE-1
#10
Leonardo C Faria, Feng Gu, Isabel Parada, Ben Barres, Z David Luo, David A Prince
The alpha2delta-1 subunit (α2δ-1) of voltage-gated calcium channels is a receptor for astrocyte-secreted thrombospondins that promote developmental synaptogenesis. Alpha2delta-1 receptors are upregulated in models of injury-induced peripheral pain and epileptogenic neocortical trauma associated with an enhancement of excitatory synaptic connectivity. These results lead to the hypothesis that overexpression of α2δ-1 alone in neocortex of uninjured transgenic (TG) mice might result in increased excitatory connectivity and consequent cortical hyperexcitability and epileptiform activity...
June 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28001087/extraction-and-purification-of-high-added-value-compounds-from-by-products-of-the-winemaking-chain-using-alternative-nonconventional-processes-technologies
#11
Sami Yammine, Sylène Brianceau, Sébastien Manteau, Mohammad Turk, Rémy Ghidossi, Eugène Vorobiev, Martine Mietton-Peuchot
Grape byproducts are today considered as a cheap source of valuable compounds since existent technologies allow the recovery of target compounds and their recycling. The goal of the current article is to explore the different recovery stages used by both conventional and alternative techniques and processes. Alternative pre-treatments techniques reviewed are: ultrasounds, pulsed electric fields and high voltage discharges. In addition, nonconventional solvent extraction under high pressure, specifically, supercritical fluid extraction and subcritical water extraction are discussed...
December 21, 2016: Critical Reviews in Food Science and Nutrition
https://www.readbyqxmd.com/read/27866201/comparative-analysis-of-gene-regulatory-network-components-in-the-auditory-hindbrain-of-mice-and-chicken
#12
COMPARATIVE STUDY
Benjamin Pawlik, Tina Schlüter, Heiner Hartwich, Saskia Breuel, Lennart Heepmann, Hans Gerd Nothwang
The neurons in the mammalian and avian auditory hindbrain nuclei share a number of significant morphological and physiological properties for fast, secure and precise neurotransmission, such as giant synapses, voltage-gated K+ channels and fast AMPA receptors. Based on the independent evolution of the middle ear in these two vertebrate lineages, on different embryonic origins of the nuclei and on marked differences on the circuit level, these similarities are assumed to reflect convergent evolution. Independent acquisition of similar phenotypes can be produced by divergent evolution of genetic mechanisms or by similar molecular mechanisms...
2016: Brain, Behavior and Evolution
https://www.readbyqxmd.com/read/27779742/gene-mutation-analysis-of-175-chinese-patients-with-early-onset-epileptic-encephalopathy
#13
Q Zhang, J Li, Y Zhao, X Bao, L Wei, J Wang
The aim of the study is to investigate the genetic characteristics and clinical features of a cohort of Chinese patients with early-onset epileptic encephalopathies (EOEEs). Targeted next-generation sequencing (NGS), focusing on 17 genes, was performed on 175 Chinese patients with EOEEs to screen gene mutations. The mutation rate was 32% (56/175). All mutations were de novo and heterozygous, including 41 novel and 15 reported mutations. Patients with cyclin-dependent kinase-like 5 (CDKL5) gene mutation accounted for the largest proportion, 13...
May 2017: Clinical Genetics
https://www.readbyqxmd.com/read/27768696/fine-mapping-of-a-dravet-syndrome-modifier-locus-on-mouse-chromosome-5-and-candidate-gene-analysis-by-rna-seq
#14
Nicole A Hawkins, Nicole J Zachwieja, Alison R Miller, Lyndsey L Anderson, Jennifer A Kearney
A substantial number of mutations have been identified in voltage-gated sodium channel genes that result in various forms of human epilepsy. SCN1A mutations result in a spectrum of severity ranging from mild febrile seizures to Dravet syndrome, an infant-onset epileptic encephalopathy. Dravet syndrome patients experience multiple seizures types that are often refractory to treatment, developmental delays, and elevated risk for SUDEP. The same sodium channel mutation can produce epilepsy phenotypes of varying clinical severity...
October 2016: PLoS Genetics
https://www.readbyqxmd.com/read/27643450/mitochondrial-porin-isoform-atvdac1-regulates-the-competence-of-arabidopsis-thaliana-to-agrobacterium-mediated-genetic-transformation
#15
Tackmin Kwon
The efficiency of Agrobacterium-mediated transformation in plants depends on the virulence of Agrobacterium strains, the plant tissue culture conditions, and the susceptibility of host plants. Understanding the molecular interactions between Agrobacterium and host plant cells is crucial when manipulating the susceptibility of recalcitrant crop plants and protecting orchard trees from crown gall disease. It was discovered that Arabidopsis voltage-dependent anion channel 1 (atvdac1) mutant has drastic effects on Agrobacterium-mediated tumorigenesis and growth developmental phenotypes, and that these effects are dependent on a Ws-0 genetic background...
September 2016: Molecules and Cells
https://www.readbyqxmd.com/read/27375106/the-scn8a-encephalopathy-mutation-p-ile1327val-displays-elevated-sensitivity-to-the-anticonvulsant-phenytoin
#16
Bryan S Barker, Matteo Ottolini, Jacy L Wagnon, Rachel M Hollander, Miriam H Meisler, Manoj K Patel
OBJECTIVE: SCN8A encephalopathy (early infantile epileptic encephalopathy; EIEE13) is caused by gain-of-function mutations resulting in hyperactivity of the voltage-gated sodium channel Nav 1.6. The channel is concentrated at the axon initial segment (AIS) and is involved in establishing neuronal excitability. Clinical features of SCN8A encephalopathy include seizure onset between 0 and 18 months of age, intellectual disability, and developmental delay. Seizures are often refractory to treatment with standard antiepileptic drugs, and sudden unexpected death in epilepsy (SUDEP) has been reported in approximately 10% of patients...
September 2016: Epilepsia
https://www.readbyqxmd.com/read/27306788/changes-in-the-proliferative-capacity-of-ng2-cell-subpopulations-during-postnatal-development-of-the-mouse-hippocampus
#17
Behrouz Moshrefi-Ravasdjani, Pavel Dublin, Gerald Seifert, Katja Jennissen, Christian Steinhäuser, Karl W Kafitz, Christine R Rose
Besides astrocytes and oligodendrocytes, NG2 proteoglycan-expressing cells (NG2 glia) represent a third subtype of macroglia in the brain. Originally described as oligodendrocyte precursor cells, they feature several characteristics not expected from mere progenitor cells, including synaptic connections with neurons. There is accumulating evidence that the properties of NG2 glia differ between different brain regions and developmental stages. To further analyze this proposed heterogeneity, we studied electrophysiological properties, transcript and protein expression, distribution and proliferative capacity of NG2 glia during postnatal development, focusing on the hippocampus and corpus callosum...
June 15, 2016: Brain Structure & Function
https://www.readbyqxmd.com/read/27210545/autosomal-dominant-scn8a-mutation-with-an-unusually-mild-phenotype
#18
G Anand, F Collett-White, A Orsini, S Thomas, S Jayapal, N Trump, Z Zaiwalla, S Jayawant
BACKGROUND: Mutations in SCN8A, coding for the voltage-gated sodium channel Nav 1.6, have been described in relation to infantile onset epilepsy with developmental delay and cognitive impairment, in particular early onset epileptic encephalopathy (EIEE) type 13. CASE REPORT: Here we report an infant and his father with early onset focal epileptic seizures but without cognitive or neurological impairment in whom next generation sequence analysis identified a heterozygous mutation (c...
September 2016: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/27065805/developmental-profile-of-ion-channel-specializations-in-the-avian-nucleus-magnocellularis
#19
Hui Hong, Lisia Rollman, Brooke Feinstein, Jason Tait Sanchez
Ultrafast and temporally precise action potentials (APs) are biophysical specializations of auditory brainstem neurons; properties necessary for encoding sound localization and communication cues. Fundamental to these specializations are voltage dependent potassium (KV) and sodium (NaV) ion channels. Here, we characterized the functional development of these ion channels and quantified how they shape AP properties in the avian cochlear nucleus magnocellularis (NM). We report that late developing NM neurons (embryonic [E] days 19-21) generate fast APs that reliably phase lock to sinusoidal inputs at 75 Hz...
2016: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/27063102/evaluation-of-microtransplantation-of-rat-brain-neurolemma-into-xenopus-laevis-oocytes-as-a-technique-to-study-the-effect-of-neurotoxicants-on-endogenous-voltage-sensitive-ion-channels
#20
Edwin Murenzi, Abigail C Toltin, Steven B Symington, Molly M Morgan, John M Clark
Microtransplantation of mammalian brain neurolemma into the plasma membrane of Xenopus oocytes is used to study ion channels in their native form as they appear in the central nervous system. Use of microtransplanted neurolemma is advantageous for various reasons: tissue can be obtained from various sources and at different developmental stages; ion channels and receptors are present in their native configuration in their proper lipid environment along with appropriate auxiliary subunits; allowing the evaluation of numerous channelpathies caused by neurotoxicants in an ex vivo state...
April 7, 2016: Neurotoxicology
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