ANIMAL MODELS OF EPILEPSY Index

Transcription

1 INDEX A Action potentials BK channel effect on See also Synaptic transmission Adeno-associated virus (AAV) vector gene therapy inhibitory GABA receptors and neuroactive peptides targets in galanin NPY Adenovirus (Ad) viral vector gene therapy , 241 Albino Xenopus laevis tadpole, see Tadpole (Xenopus laevis) seizure model Aldicarb assay in C. elegans model See also PTZ (pentylenetetrazole) Amino acid receptors, see Excitatory amino acid receptors Antiepileptic drugs (AEDs) for seizure-sensitive mutants in developing zebrafish See also Viral vector gene therapy; Topoisomerase I (top1) inhibitors Anti-seizure treatments AED top1 inhibitors Arx mutations in interneuron-deficient mice See also Dlx family mutations Ataxia...91, 94 Auditory hair cells Autism Axonal sprouting BDNF affected kainic acid (KA)-induced mossy fiber (MFS) , See also Epileptogenesis B Bang-sensitive (BS) mutants seizure-sensitive mutant See also suppressor mutations under Seizures Behavioral assays strategy for isolating epilepsy genes and susceptibility factors with C. elegans model defecation assay to identify excitatory GABA signaling molecules foraging, locomotion, and shrinking assays to identify inhibitory GABA signaling molecules See also Bioinformatics; Electrophysiological assays; Pharmacological assays Behavioral seizures in X. laevis tadpoles chemoconvulsant-induced behavioral seizures C-shaped contraction See also Tadpole (Xenopus laevis) seizure model Benign familial neonatal convulsions (BFNC) mouse models dominant-negative KCNQ2 G279S KCNQ2 knockin mouse KCNQ2 knockout mouse KCNQ3 knockin mouse Szt1 spontaneous C-terminal deletion mutation mutations in KCNQ (Kv7) genes encoding M channel subunits KCNQ2 (Kv7.2), KCNQ3 (Kv7.3), M current role in neuron physiology mouse models Bioinformatics strategy for isolating epilepsy genes and susceptibility factors with C. elegans model , 27 See also Behavioral assays; Electrophysiological assays; Pharmacological assays BK channels effect on action potentials effect on synaptic transmission firing frequency and dentate gyrus function mutations mutations causing epilepsy KCNMA KCNMB KCNMB mutations causing loss of function ataxia and defects in cerebellar function...91, 94 auditory hair cells changes in circadian rhythms haploinsufficiency and autism KCNMA1...91, 94 See also M type potassium channel (M channel) 265

2 266 ANIMAL MODELS OF EPILEPSY Brain derived neurotrophic factor (BDNF) affected axonal sprouting See also Epileptogenesis Brain development tadpole seizure model zebrafish seizure model , 62 C Caenorhabditis elegans seizure model features gross organization of nervous system synapses characteristics... 6 model for general human biology nervous system strategies for isolating epilepsy genes and susceptibility factors behavioral screening bioinformatics , 27 electrophysiological methods pharmacological assays synaptic transmission genes... 3, 4 See also Drosophila melanogaster seizure model; Tadpole (Xenopus laevis) seizure model; Zebrafish (Danio rerio) seizure model Calcium activated potassium channel, see BK channels sensitive dyes Calcium (Ca 2+ ) imaging and electrophysiology to guide imaging to interpret imaging using imaging to guide electrophysiology in X. laevis tadpoles See also In vitro imaging; In vivo imaging; Magnesium (Mg 2+ ) imaging; Optical imaging Calretinin (CR) Camptothecin (CPT) See also Topoisomerase I (top1) inhibitors CD44 expression axonal sprouting and See also Organotypic hippocampal slice cultures Chirps as models of epileptiform events See also Electrophysiological assays; Seizure detection Circadian rhythms changes in See also BK channels Classifiers defined pattern classification...217, See also Feature extraction; Seizure detection CMV promoter Computational models approaches hyperexcitable dentate network large-scale dentate gyrus model visualization See also Dentate gyrus model; Seizure models Cortical maps importance of See also In vitro imaging Cortical tuber C-shaped contraction See also Tadpole (Xenopus laevis) seizure model Cyclin D2 (cd2) mutations interneuron-deficient mice and See also Dlx family mutations D D434G KCNMA1, see under KCNMA1 gene mutations Danio rerio, see Zebrafish (Danio rerio) seizure model Defecation assay to identify excitatory GABA signaling molecules motor program (DMP) Dentate granule cells (DGCs) ectopic DGCs in mtle...252, firing frequency and DG function in BK channels functional implications of seizure-induced neurogenesis mammalian hippocampal neurogenesis and See also Hilar basal dendrites (HBD) Dentate gyrus model large scale development for epileptogenesis topological determinants study functional implications of structural alterations robustness utility in epilepsy research visualization...177, 179 neuronal hubs promoting hyperexcitability in structural 1:1 scale model See also Seizure models Developmental seizures model tadpole, see Tadpole (Xenopus laevis) seizure model zebrafish AEDs evaluation seizures monitoring methods See also Computational models; Seizure models Dlx family mutations Dlx1 and Dlx

3 ANIMAL MODELS OF EPILEPSY 267 non-embryonic lethal (Dlx1 / ) See also Interneurons-deficient mice Drosophila melanogaster seizure model anti-seizure treatments AED treatment drug treatments top1 inhibitors for human epilepsy seizure-sensitive mutants anti-seizure treatments for BS mutants sda mutants...29, 31 using Drosophila for seizure studies DNA topoisomerase I mutations gap junction mutations seizure-like activity...29, 32 seizure-like electrical activity , 32 seizure-sensitive mutants seizure-suppressor mutations sodium channel mutations See also Caenorhabditis elegans seizure model; Tadpole (Xenopus laevis) seizure model; Zebrafish (Danio rerio) seizure model Drug screening model See also Organotypic hippocampal slice cultures E Electroencephalography (EEG) epileptiform intracranial (IEEG)...210, scalp See also Electrophysiology; In vitro imaging; In vivo imaging Electrophysiological assays strategy for isolating epilepsy genes and susceptibility factors with C. elegans model in vitro electrophysiological methods in vivo electrophysiological methods optical imaging See also Behavioral assays; Bioinformatics; Pharmacological assays Electrophysiology for seizures analysis in seizure model tadpole zebrafish hippocampal slice cultures as epileptogenesis model and to guide imaging to interpret imaging using imaging to guide electrophysiology See also Electroencephalography (EEG) Embryonic lethal interneuron-deficient mice, see under Interneurons-deficient mice Enhancers See also suppressor mutations under Seizures Epilepsy BK channels mutations causing KCNMA KCNMB KCNMB computational model (large scale dentate gyrus model) for epileptogenesis topological determinants study utility in epilepsy research exogenous stem cells in GABA signaling and in vitro models interneuron-deficient mice relevance to mesial temporal lobe (mtle) models usefulness strategies for isolating epilepsy genes and susceptibility factors with C. elegans model behavioral screening bioinformatics , 27 electrophysiological methods pharmacological assays temporal lobe viral vector gene therapy for See also Epileptogenesis; Hippocampal neurogenesis; Seizure models Epileptiform EEG application examples chirps as models of epileptiform events line length in seizure prediction with trending and feature composition spread rapidly propagating slowly propagating See also Electrophysiology; Electrophysiological assays; In vitro imaging Epileptogenesis associated axonal rearrangements kainic acid-induced sprouting mossy fiber sprouting defined organotypic hippocampal slice cultures for epileptogenesis-associated axonal rearrangements study model See also Epilepsy; Hippocampal neurogenesis; Seizure models Excitatory amino acid receptors inhibitory GABA receptors and NMDA See also Viral vector gene therapy

4 268 ANIMAL MODELS OF EPILEPSY Excitatory GABA receptors defecation assay to identify excitatory GABA signaling molecules expulsion defective phenotype See also Inhibitory GABA receptors Expulsion defective phenotype defined F Feature composition EEG epileptiform and See also Electroencephalography (EEG); Seizure detection Feature extraction feature definition in in vivo seizure detection See also Classifiers Firing frequency and dentate gyrus function See also BK channels Fluorescent microscopy for tadpole seizure activity study See also In vitro imaging; In vivo imaging Foraging in C. elegans... 8 See also GABA signaling G GABA signaling epilepsy and excitatory exogenous stem cells in mtle and foraging and... 8 GABAergic interneuron development diversity inhibitory See also Interneurons-deficient mice GAD interneurons exogenous stem cells in mtle and Galanin for viral vector gene therapy See also Neuropeptide Y (NPY) Ganglionic eminence (GE) caudal (CGE) lateral (LGE) medial (MGE) See also Interneurons Gap junction mutations as seizure supressors See also Sodium channel mutations; Topoisomerase I (top1 JS ) mutations Gene therapy, see Viral vector gene therapy GENESIS program See also Computational models Glial cell line-derived neurotrophic factor (GDNF) viral vector gene therapy and , 241 H Hair cells, auditory Hamartomas Haploinsufficiency and autism See also BK channels Hepatocyte growth factor (HGF) Herpes simplex virus (HSV) viral vector gene therapy Hilar basal dendrites (HBD) in mtle See also See also Dentate granule cells (DGCs); Mossy fiber sprouting (MBS) Hippocampal neurogenesis adult mammalian altered neurogenesis in mtle ectopic DGCs functional implications of seizure-induced neurogenesis hilar basal dendrites mossy fiber sprouting Hippocampal sclerosis Hippocampal slice cultures, see Organotypic hippocampal slice cultures Hubs promoting hyperexcitability neuronal See also Dentate gyrus model Human immunodeficiency virus (HIV) viral vector gene therapy Hyperexcitability I ICP10 PK expression Ictal See also In vitro imaging; In vivo imaging Immature brain seizures in immature zebrafish See also Zebrafish (Danio rerio) seizure model In vitro imaging... 19, 141 calcium (Ca 2+ ) epileptiform activity rapidly propagating slowly propagating for zebrafish seizures monitoring methods future research usefulness of epilepsy models See also Cortical maps; Electrophysiology; In vivo imaging

5 ANIMAL MODELS OF EPILEPSY 269 In vivo imaging in X. laevis tadpoles imaging of seizure activity circuit properties using calcium-sensitive dyes imaging of seizure-induced effects on neuronal development in zebrafish seizure detection analysis classifiers training experimental setup feature extraction general detection frameworks , 212 recordings considerations See also Electrophysiology; In vitro imaging Inhibitory GABA receptors foraging assays... 8 in viral vector gene therapy locomotion assays shrinker phenotype See also Excitatory GABA receptors Interface method See also Organotypic hippocampal slice cultures Interictal See also Seizure detection Interneurons (GABAergic) development diversity Interneurons-deficient mice embryonic lethal Arx gene Dlx family (Dlx1, 2, and Dlx5) Nkx epilepsy and non-embryonic lethal cd Dlx family (Dlx1 / ) MET Ppt Tlx / upar utility of Intracranial EEG (IEEG)...203, 210, 213, 215 See also Seizure detection J Joint sign periodogram event characterization transform (JSPECT) , 226 See also Epileptiform K Kainic acid (KA) induced axonal sprouting See also Epileptogenesis KCNMA1 gene mutations BK channels mutations causing loss of function ataxia and defects in cerebellar function...91, 94 auditory hair cells changes in circadian rhythms D434G gain of function of BK channels mutations causing epilepsy KCNMB3 gene mutations KCNMB4 gene knockout KCNQ genes mutations KCNQ2 (Kv7.2) dominant-negative KCNQ2 G279S encoding M channel subunits knockin mouse knockout mouse M current role in neuron physiology KCNQ3 (Kv7.3) encoding M channel subunits knockin mouse M current role in neuron physiology KCNQ Szt1 spontaneous C-terminal deletion mutation See also Benign familial neonatal convulsions (BFNC) Knock-in, see under Mouse models Knock-out, see under Mouse models Kv7 gene, see KCNQ genes mutations L Large-scale dentate gyrus model, see under Dentate gyrus model Line length (LL) defined See also Seizure detection LIS Locomotion... 9 GABA signaling and in C. elegans... 8 M M type potassium channel (M channel) KCNQ genes mutations and genes encoding M channel subunits M current role in neuron physiology See also Benign familial neonatal convulsions (BFNC); BK channels Magnesium (Mg 2+ ) imaging epileptiform spread and 0 Mg 2+ model See also Calcium (Ca 2+ ) imaging; In vitro imaging MaxiK channels, see BK channels

6 270 ANIMAL MODELS OF EPILEPSY Mesial temporal lobe epilepsy (mle) altered neurogenesis in ectopic DGCs functional implications of seizure-induced neurogenesis hilar basal dendrites mossy fiber sprouting exogenous stem cells in neural stem cells (NSCs) in MET mutations mle napts seizure-suppressor mutants Mossy fiber sprouting (MFS) in mtle in organotypic hippocampal slice cultures See also Dentate granule cells (DGCs); Epileptogenesis; Hilar basal dendrites (HBD) Mouse models KCNQ genes mutations knock-in KCNQ KCNQ TSC model knock-out interneuron-deficient KCNQ TSC rodent model TSC conditional conventional See also Seizure models mtor (mammalian Target Of Rapamycin)... 78, 83 N Nematode seizure model, see Caenorhabditis elegans seizure model Neonatal convulsions, see Benign familial neonatal convulsions (BFNC) Neural stem cells (NSCs) exogenous stem cells in epilepsy in mtle Neuroactive peptides galanin neuropeptide Y (NPY) See also Viral vector gene therapy Neurogenesis, see Hippocampal neurogenesis NEURON program See also Computational models Neuronal development in X. laevis tadpoles (in vivo imaging) hubs promoting hyperexcitability in dentate gyrus model Neuropeptide Y (NPY) for viral vector gene therapy See also Galanin Nkx2.1 gene mutation See also Interneurons-deficient mice N-methyl-D-aspartic acid (NMDA) receptors antagonist and hippocampal slice cultures receptor 1 protein (NMDAR1), 237 See also Viral vector gene therapy O Optical imaging for C. elegans seizure model for zebrafish seizures monitoring methods See also In vitro imaging; In vivo imaging Organotypic hippocampal slice cultures as drug screening model as epileptogenesis model for epileptogenesis-associated axonal rearrangements study kainic acid-induced sprouting mossy fiber sprouting preparation amd maintenance comparison between methods interface method roller tube method properties P Palmitoyl protein thioesterase 1 (Ppt1) mutations para st76 seizure-suppressor mutants Parvalbumin (PV) Pharmacological assays aldicarb assay PTZ assay See also Behavioral assays; Bioinformatics; Electrophysiological assays Potassium channel, see BK channels; M type potassium channel (M channel) PTZ (pentylenetetrazole) X. laevis tadpoles model behavioral seizures study electrographic seizures study zebrafish seizure model and , See also Aldicarb assay R Rapamycin, see mtor (mammalian Target Of Rapamycin) Recovery seizure Roller tube method

7 ANIMAL MODELS OF EPILEPSY 271 S Scatter factor, see Hepatocyte growth factor (HGF) Seizure detection epileptiform EEG feature extraction and pattern classification in vitro calcium (Ca 2+ ), epileptiform activity for zebrafish seizures monitoring methods future research usefulness of epilepsy models in vivo classifiers training experimental setup feature extraction general detection frameworks , 212 recordings considerations need for real-time seizure detection in therapeutic and/or diagnostic devices practical methods See also Seizures Seizure models Caenorhabditis elegans Drosophila melanogaster Xenopus laevis (tadpole) Danio rerio (zebrafish) See also Computational models; Tuberous sclerosis complex (TSC) model Seizures coma paralytic period developmental tadpole (Xenopus laevis) seizure model zebrafish electrophysiological analysis genetic interactions enhancement suppression detection, see Seizure detection interneuron loss and 121, see also under Interneuronsdeficient mice models, see Seizure models recovery seizure refractory period sensitive mutants bang-sensitive (BS) sda suppressor mutations DNA topoisomerase I gap junction sodium channel See also Epileptogenesis; Viral vector gene therapy shakb 2 seizure-suppressor mutants Shrinker phenotype Single-cell electroporation (SCE)... 53, 55 Slamdance (sda) mutants... 29, 31 Slice cultures, see Organotypic hippocampal slice cultures Sodium channel mutations as seizure supressor See also Gap junction mutations; Topoisomerase I (top1 JS ) mutation Somatostatin (SOM) Sox2 knockdown mouse Sprouting, see Axonal sprouting Stem cells, see Neural stem cells (NSCs) Structural 1:1 scale model, see under Dentate gyrus model Subependymal giant cell astrocytomas (SEGAs) Suppressor mutations, see under Seizures Synaptic transmission BK channel effect on in C. elegans , 6 T Tadpole (Xenopus laevis) seizure model as new developmental seizures model experimental model of early brain development in vivo imaging of seizure-induced effects on neuronal development seizure activity study techniques behavioral seizures electrographic seizure activity in vivo imaging of seizure activity circuit properties using calcium-sensitive dyes See also Caenorhabditis elegans seizure model; Drosophila melanogaster seizure model; Zebrafish (Danio rerio) seizure model Temporal lobe epilepsy (TLE) , 188 mesial, see Mesial temporal lobe epilepsy (mle) viral vector gene therapy for See also Epileptogenesis Tlx / mutations See also Interneurons-deficient mice Topoisomerase I (top1) inhibitors See also Antiepileptic drugs (AEDs) Topoisomerase I (top1 JS ) mutations anti-seizure treatments and suppression by top1 JS as seizure-suppressor See also Gap junction mutations; Sodium channel mutations Tuberous sclerosis complex (TSC) clinical features future scope genetics

8 272 ANIMAL MODELS OF EPILEPSY Tuberous sclerosis (continued) TSC1 mutations TSC2 mutations models knock-in knock-out neuropathology in neuropathology in human signaling pathway TSC1 conditional mouse models conventional rodent models of TSC2 (conventional rodent models) zebrafish seizure model of Tubers See also Tuberous sclerosis complex (TSC) Two-photon microscopy in vivo See also In vitro imaging; In vivo imaging U upar (urokinase plasminogen activator receptor) mutations V Viral vector gene therapy adeno-associated virus (AAV) adenovirus (Ad) critical considerations and future directions excitatory amino acid receptors in (NMDA) HIV HSV neuroactive peptides (NPY and galanin) potential therapeutic targets excitatory amino acid receptors identification of inhibitory GABA receptors See also Antiepileptic drugs (AEDs) Voltage-sensitive dye See also Calcium (Ca 2+ ) imaging X Xenopus laevis, see Tadpole (Xenopus laevis) seizure model Z Zebrafish (Danio rerio) seizure model of TSC seizures in developing zebrafish AEDs evaluation developing zebrafish brain organization seizures monitoring methods stage I-III zebrafish as model organism See also Caenorhabditis elegans seizure model; Drosophila melanogaster seizure model; Tadpole (Xenopus laevis) seizure model