Research Topics
Species | Jeffrey NoebelsSummaryAffiliation: Baylor College of Medicine Country: USA Publications
Research Grants
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Detail Information
Publications
Expanded alternative splice isoform profiling of the mouse Cav3.1/alpha1G T-type calcium channelWayne L Ernst
Baylor College of Medicine, Houston, TX 77030, USA
BMC Mol Biol 10:53. 2009....- The Judith Hoyer Lecture: genes, pixels, patterns, and preventionJeffrey L Noebels
Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Epilepsy Behav 9:379-85. 2006 
Exploring new gene discoveries in idiopathic generalized epilepsyJeffrey L Noebels
Developmental Neurogenetics Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, U S A
Epilepsia 44:16-21. 2003..Further analyses of these gene mutations and their effects on the developing brain are providing critical clues in the search to explain the origin of "idiopathic" epilepsy...- The biology of epilepsy genesJeffrey L Noebels
Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Annu Rev Neurosci 26:599-625. 2003..This review selects exemplary members of several gene families to illustrate principal categories of the disease and trace the biological pathways to epileptogenesis in the developing brain... - Modeling human epilepsies in miceJ L Noebels
Blue Bird Circle Developmental Neurogenetics Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
Epilepsia 42:11-5. 2001..Along with providing a unique opportunity to understand the mechanisms of inherited epileptogenesis, the mouse models serve as ideal biological test systems to search for novel therapeutic strategies... - Altered ultrasonic vocalization and impaired learning and memory in Angelman syndrome mouse model with a large maternal deletion from Ube3a to Gabrb3Yong hui Jiang
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
PLoS ONE 5:e12278. 2010..These mice will be valuable for future comparative studies to mice with maternal deficiency of Ube3a alone... - A perfect storm: Converging paths of epilepsy and Alzheimer's dementia intersect in the hippocampal formationJeffrey Noebels
Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
Epilepsia 52:39-46. 2011.... - Exome sequencing of ion channel genes reveals complex profiles confounding personal risk assessment in epilepsyTara Klassen
Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA
Cell 145:1036-48. 2011.... - Ablation of steroid receptor coactivator-3 resembles the human CACT metabolic myopathyBrian York
Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX 77030, USA
Cell Metab 15:752-63. 2012..Moreover, these findings allow us to consider platform coactivators such as the SRCs as potential contributors to syndromes such as CACT deficiency, previously considered as monogenic... - Rai1 deficiency in mice causes learning impairment and motor dysfunction, whereas Rai1 heterozygous mice display minimal behavioral phenotypesWeimin Bi
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030 3498, USA
Hum Mol Genet 16:1802-13. 2007.... - Epilepsy and chromosomal rearrangements in Smith-Magenis Syndrome [del(17)(p11.2p11.2)]Alica M Goldman
Department of Neurology, Baylor College of Medicine, Texas Children s Hospital, Houston, TX 77030, USA
J Child Neurol 21:93-8. 2006.... - Aberrant excitatory neuronal activity and compensatory remodeling of inhibitory hippocampal circuits in mouse models of Alzheimer's diseaseJorge J Palop
Gladstone Institute of Neurological Disease, San Francisco, CA 94158, USA
Neuron 55:697-711. 2007..Aberrant increases in network excitability and compensatory inhibitory mechanisms in the hippocampus may contribute to Abeta-induced neurological deficits in hAPP mice and, possibly, also in humans with AD... - Masking epilepsy by combining two epilepsy genesEdward Glasscock
Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, Texas 77030, USA
Nat Neurosci 10:1554-8. 2007.... - Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3Rebecca P Seal
Department of Physiology, School of Medicine, University of California, San Francisco, San Francisco, CA 94143, USA
Neuron 57:263-75. 2008..The glutamate release conferred by expression of VGLUT3 thus has an essential role in both function and development of the auditory pathway, as well as in the control of cortical excitability... - 2006 Merritt Putnam Symposium: mapping epileptic circuitryJeffrey L Noebels
Department of Neurology, Baylor College of Medicine, Houston, Texas, U.S.A
Epilepsia 49:1-2. 2008 - Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganizationNanda A Singh
Department of Human Genetics, University of Utah, Salt Lake City, UT 84112, USA
J Physiol 586:3405-23. 2008..The absence of seizure-induced pathology found in these epileptic mouse models parallels the benign neurodevelopmental cognitive profile exhibited by the majority of BFNC patients... - A mouse model for Glut-1 haploinsufficiencyDong Wang
Colleen Giblin Laboratories for Pediatric Neurology Research, Department of Neurology, Columbia University, New York, NY 10032, USA
Hum Mol Genet 15:1169-79. 2006..This GLUT-1+/- mouse model creates an opportunity to investigate Glut-1 function, to examine the pathophysiology of Glut-1 DS in vivo and to evaluate new treatment strategies... - BK channel beta4 subunit reduces dentate gyrus excitability and protects against temporal lobe seizuresRobert Brenner
Department of Physiology, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, Texas 78229, USA
Nat Neurosci 8:1752-9. 2005.... - Mice lacking Dlx1 show subtype-specific loss of interneurons, reduced inhibition and epilepsyInma Cobos
Nina Ireland Laboratory of Developmental Neurobiology, Department of Psychiatry, University of California San Francisco, San Francisco, California 94158, USA
Nat Neurosci 8:1059-68. 2005..Dlx1 mutant mice show generalized electrographic seizures and histological evidence of seizure-induced reorganization, linking the Dlx1 mutation to delayed-onset epilepsy associated with interneuron loss... - Mutations in high-voltage-activated calcium channel genes stimulate low-voltage-activated currents in mouse thalamic relay neuronsYi Zhang
Developmental Neurogenetics Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
J Neurosci 22:6362-71. 2002..These alterations increase the probability for abnormal thalamocortical synchronization and absence epilepsy in tg, lh, and stg mice... - Genetic disruption of cortical interneuron development causes region- and GABA cell type-specific deficits, epilepsy, and behavioral dysfunctionElizabeth M Powell
Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Neurosci 23:622-31. 2003.... - Mice lacking sodium channel beta1 subunits display defects in neuronal excitability, sodium channel expression, and nodal architectureChunling Chen
Department of Pharmacology, The University of Michigan, Ann Arbor, Michigan 48109 0632, USA
J Neurosci 24:4030-42. 2004.... - Elevated thalamic low-voltage-activated currents precede the onset of absence epilepsy in the SNAP25-deficient mouse mutant colobomaYi Zhang
Developmental Neurogenetics Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
J Neurosci 24:5239-48. 2004..We suggest that presynaptic release defects shared by these mutants lead to postsynaptic LVA excitability increases in thalamic pacemaker neurons that favor rebound bursting and absence epilepsy... - Sodium channel gene expression and epilepsyJeffrey L Noebels
Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA
Novartis Found Symp 241:109-20; discussion 120-3, 226-32. 2002..Analysis of these models offers insight into developmental processes that control the cellular expression and plasticity of Na+ channel genes, and will help to clarify mechanisms of hereditary Na+ channel-based epileptogenesis... - Neuronal LRP1 functionally associates with postsynaptic proteins and is required for normal motor function in micePetra May
Zentrum für Neurowissenschaften, University of Freiburg, Albertstrabetae 23, 79104 Freiburg, Germany
Mol Cell Biol 24:8872-83. 2004..Together, these findings suggest that LRP1, like other ApoE receptors, can modulate synaptic transmission in the brain... - Genetic mouse models of essential tremor: are they essential?Joseph Jankovic
Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
J Clin Invest 115:584-6. 2005.... - Visualization of transmitter release with zinc fluorescence detection at the mouse hippocampal mossy fibre synapseJing Qian
Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
J Physiol 566:747-58. 2005..The ability to directly visualize release dynamics with zinc imaging will facilitate the exploration of the molecular pharmacology and plasticity of exocytosis at MF synapses... - Models for epilepsy and epileptogenesis: report from the NIH workshop, Bethesda, MarylandJames P Stables
National Institute of Neurological Disorders and Stroke, Bethesda, Maryland 20892, USA
Epilepsia 43:1410-20. 2002..To move toward new and more effective therapies, novel approaches to therapy discovery are needed... - Exocytosis of vesicular zinc reveals persistent depression of neurotransmitter release during metabotropic glutamate receptor long-term depression at the hippocampal CA3-CA1 synapseJing Qian
Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
J Neurosci 26:6089-95. 2006..The ability to examine release dynamics with zinc fluorescence detection will facilitate exploration of the molecular pharmacology and plasticity of exocytosis at many CNS synapses... - Genetic and phenotypic analysis of the mouse mutant mh2J, an Ap3d allele caused by IAP element insertionPrameela Kantheti
Mental Health Research Inst, University of Michigan, 205 Zina Pitcher Place, Ann Arbor 48109 0720, USA
Mamm Genome 14:157-67. 2003.... - 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 imbalanceKatherina Walz
Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Mol Cell Biol 23:3646-55. 2003..Our murine models represent a powerful tool to analyze the consequences of gene dosage imbalance in this genomic interval and to investigate the molecular genetic bases of both SMS and dup(17)(p11.2p11.2)... - Topiramate alters excitatory synaptic transmission in mouse hippocampusJing Qian
Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Epilepsy Res 55:225-33. 2003..In addition, the ability of TPM to reduce postsynaptic Ca2+ buildup may provide a potential mechanism for neuronal protection during paroxysmal firing associated with epileptic seizures... - Sodium channel Scn1b null mice exhibit prolonged QT and RR intervalsLuis F Lopez-Santiago
Department of Pharmacology, University of Michigan, 1301 MSRB III, Ann Arbor, MI 48109 0632, USA
J Mol Cell Cardiol 43:636-47. 2007..Together, these results suggest that beta1 is critical for normal cardiac excitability and loss of beta1 may be associated with a long QT phenotype... - Mild overexpression of MeCP2 causes a progressive neurological disorder in miceAnn L Collins
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
Hum Mol Genet 13:2679-89. 2004..Furthermore, these results support the possibility that duplications or gain-of-function mutations in MECP2 might underlie some cases of X-linked delayed-onset neurobehavioral disorders... - Genetics of epilepsy: epilepsy research foundation workshop reportSanjay Sisodiya
Epilepsy Research Foundation, United Kingdom
Epileptic Disord 9:194-236. 2007..Presentations and their matched discussions are produced here. There was optimism that further genetic research in epilepsy was not only feasible, but might lead to improvements in the lives of people with epilepsy...
Research Grants
- NEUROPHYSIOLOGY DATABASE OF INBRED MUTANT STRAINSJeffrey Noebels; Fiscal Year: 2001..This project will provide the framework for normative data to be used for comparative electrophysiological, behavioral and experimental pharmacology on inbred mouse strains and defined gene mutations. ..
- Course Development in the Neurobiology of DiseaseJeffrey Noebels; Fiscal Year: 2006....
- Parallel Sequence Profiling of Ion Channels in EpilepsyJeffrey Noebels; Fiscal Year: 2007..abstract_text> ..
- Excitability and Plasticity in Epileptic BrainJeffrey Noebels; Fiscal Year: 2006....
- Excitability and Plasticity in Developing Epileptic BrainJeffrey Noebels; Fiscal Year: 2007..Gene interactions that may prevent seizures from appearing are also explored, and may help predict the risk of childhood epilepsy. ..
- PLASTICITY IN DEVELOPING EPILEPTIC BRAINJeffrey Noebels; Fiscal Year: 1993....
- PLASTICITY IN DEVELOPING EPILEPTIC BRAINJeffrey Noebels; Fiscal Year: 2001..These studies will directly test key hypotheses concerning basic mechanisms of generalized epilepsy, and the degree of long-term cellular and molecular neuroplasticity that may accompany early seizures of the spike-wave pattern. ..