Jeffrey Noebels

Summary

Affiliation: Texas Medical Center
Country: USA

Publications

  1. pmc Expanded alternative splice isoform profiling of the mouse Cav3.1/alpha1G T-type calcium channel
    Wayne L Ernst
    Baylor College of Medicine, Houston, TX 77030, USA
    BMC Mol Biol 10:53. 2009
  2. pmc The Judith Hoyer Lecture: genes, pixels, patterns, and prevention
    Jeffrey L Noebels
    Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Epilepsy Behav 9:379-85. 2006
  3. ncbi request reprint Exploring new gene discoveries in idiopathic generalized epilepsy
    Jeffrey L Noebels
    Developmental Neurogenetics Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, U S A
    Epilepsia 44:16-21. 2003
  4. ncbi request reprint The biology of epilepsy genes
    Jeffrey L Noebels
    Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Annu Rev Neurosci 26:599-625. 2003
  5. ncbi request reprint Modeling human epilepsies in mice
    J L Noebels
    Blue Bird Circle Developmental Neurogenetics Laboratory, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
    Epilepsia 42:11-5. 2001
  6. pmc Altered ultrasonic vocalization and impaired learning and memory in Angelman syndrome mouse model with a large maternal deletion from Ube3a to Gabrb3
    Yong hui Jiang
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
    PLoS ONE 5:e12278. 2010
  7. pmc A perfect storm: Converging paths of epilepsy and Alzheimer's dementia intersect in the hippocampal formation
    Jeffrey Noebels
    Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
    Epilepsia 52:39-46. 2011
  8. pmc Exome sequencing of ion channel genes reveals complex profiles confounding personal risk assessment in epilepsy
    Tara Klassen
    Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA
    Cell 145:1036-48. 2011
  9. pmc Ablation of steroid receptor coactivator-3 resembles the human CACT metabolic myopathy
    Brian York
    Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX 77030, USA
    Cell Metab 15:752-63. 2012
  10. ncbi request reprint Rai1 deficiency in mice causes learning impairment and motor dysfunction, whereas Rai1 heterozygous mice display minimal behavioral phenotypes
    Weimin Bi
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030 3498, USA
    Hum Mol Genet 16:1802-13. 2007

Research Grants

  1. NEUROPHYSIOLOGY DATABASE OF INBRED MUTANT STRAINS
    Jeffrey Noebels; Fiscal Year: 2001
  2. Course Development in the Neurobiology of Disease
    Jeffrey Noebels; Fiscal Year: 2006
  3. Parallel Sequence Profiling of Ion Channels in Epilepsy
    Jeffrey Noebels; Fiscal Year: 2007
  4. Excitability and Plasticity in Epileptic Brain
    Jeffrey Noebels; Fiscal Year: 2006
  5. Excitability and Plasticity in Developing Epileptic Brain
    Jeffrey Noebels; Fiscal Year: 2007
  6. Excitability and Plasticity in Developing Epileptic Brain
    Jeffrey Noebels; Fiscal Year: 2009
  7. PLASTICITY IN DEVELOPING EPILEPTIC BRAIN
    Jeffrey Noebels; Fiscal Year: 1993
  8. PLASTICITY IN DEVELOPING EPILEPTIC BRAIN
    Jeffrey Noebels; Fiscal Year: 2001

Detail Information

Publications35

  1. pmc Expanded alternative splice isoform profiling of the mouse Cav3.1/alpha1G T-type calcium channel
    Wayne L Ernst
    Baylor College of Medicine, Houston, TX 77030, USA
    BMC Mol Biol 10:53. 2009
    ....
  2. pmc The Judith Hoyer Lecture: genes, pixels, patterns, and prevention
    Jeffrey L Noebels
    Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Epilepsy Behav 9:379-85. 2006
  3. ncbi request reprint Exploring new gene discoveries in idiopathic generalized epilepsy
    Jeffrey 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...
  4. ncbi request reprint The biology of epilepsy genes
    Jeffrey 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...
  5. ncbi request reprint Modeling human epilepsies in mice
    J 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...
  6. pmc Altered ultrasonic vocalization and impaired learning and memory in Angelman syndrome mouse model with a large maternal deletion from Ube3a to Gabrb3
    Yong 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...
  7. pmc A perfect storm: Converging paths of epilepsy and Alzheimer's dementia intersect in the hippocampal formation
    Jeffrey Noebels
    Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA
    Epilepsia 52:39-46. 2011
    ....
  8. pmc Exome sequencing of ion channel genes reveals complex profiles confounding personal risk assessment in epilepsy
    Tara Klassen
    Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA
    Cell 145:1036-48. 2011
    ....
  9. pmc Ablation of steroid receptor coactivator-3 resembles the human CACT metabolic myopathy
    Brian 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...
  10. ncbi request reprint Rai1 deficiency in mice causes learning impairment and motor dysfunction, whereas Rai1 heterozygous mice display minimal behavioral phenotypes
    Weimin Bi
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030 3498, USA
    Hum Mol Genet 16:1802-13. 2007
    ....
  11. ncbi request reprint 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
    ....
  12. ncbi request reprint Aberrant excitatory neuronal activity and compensatory remodeling of inhibitory hippocampal circuits in mouse models of Alzheimer's disease
    Jorge 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...
  13. ncbi request reprint Masking epilepsy by combining two epilepsy genes
    Edward Glasscock
    Department of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, Texas 77030, USA
    Nat Neurosci 10:1554-8. 2007
    ....
  14. pmc Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3
    Rebecca 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...
  15. doi request reprint 2006 Merritt Putnam Symposium: mapping epileptic circuitry
    Jeffrey L Noebels
    Department of Neurology, Baylor College of Medicine, Houston, Texas, U S A
    Epilepsia 49:1-2. 2008
  16. pmc Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganization
    Nanda 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...
  17. ncbi request reprint A mouse model for Glut-1 haploinsufficiency
    Dong 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...
  18. ncbi request reprint BK channel beta4 subunit reduces dentate gyrus excitability and protects against temporal lobe seizures
    Robert 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
    ....
  19. ncbi request reprint Mice lacking Dlx1 show subtype-specific loss of interneurons, reduced inhibition and epilepsy
    Inma 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...
  20. ncbi request reprint Mutations in high-voltage-activated calcium channel genes stimulate low-voltage-activated currents in mouse thalamic relay neurons
    Yi 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...
  21. ncbi request reprint Genetic disruption of cortical interneuron development causes region- and GABA cell type-specific deficits, epilepsy, and behavioral dysfunction
    Elizabeth M Powell
    Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
    J Neurosci 23:622-31. 2003
    ....
  22. ncbi request reprint Mice lacking sodium channel beta1 subunits display defects in neuronal excitability, sodium channel expression, and nodal architecture
    Chunling Chen
    Department of Pharmacology, The University of Michigan, Ann Arbor, Michigan 48109 0632, USA
    J Neurosci 24:4030-42. 2004
    ....
  23. ncbi request reprint Elevated thalamic low-voltage-activated currents precede the onset of absence epilepsy in the SNAP25-deficient mouse mutant coloboma
    Yi 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...
  24. ncbi request reprint Sodium channel gene expression and epilepsy
    Jeffrey 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...
  25. pmc Neuronal LRP1 functionally associates with postsynaptic proteins and is required for normal motor function in mice
    Petra 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...
  26. pmc 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
    ....
  27. pmc Visualization of transmitter release with zinc fluorescence detection at the mouse hippocampal mossy fibre synapse
    Jing 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...
  28. ncbi request reprint Models for epilepsy and epileptogenesis: report from the NIH workshop, Bethesda, Maryland
    James 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...
  29. ncbi request reprint Exocytosis of vesicular zinc reveals persistent depression of neurotransmitter release during metabotropic glutamate receptor long-term depression at the hippocampal CA3-CA1 synapse
    Jing 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...
  30. ncbi request reprint Genetic and phenotypic analysis of the mouse mutant mh2J, an Ap3d allele caused by IAP element insertion
    Prameela Kantheti
    Mental Health Research Inst, University of Michigan, 205 Zina Pitcher Place, Ann Arbor 48109 0720, USA
    Mamm Genome 14:157-67. 2003
    ....
  31. pmc 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 imbalance
    Katherina 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)...
  32. ncbi request reprint Topiramate alters excitatory synaptic transmission in mouse hippocampus
    Jing 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...
  33. pmc Sodium channel Scn1b null mice exhibit prolonged QT and RR intervals
    Luis 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...
  34. ncbi request reprint Mild overexpression of MeCP2 causes a progressive neurological disorder in mice
    Ann 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...
  35. ncbi request reprint Genetics of epilepsy: epilepsy research foundation workshop report
    Sanjay 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 Grants29

  1. NEUROPHYSIOLOGY DATABASE OF INBRED MUTANT STRAINS
    Jeffrey 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. ..
  2. Course Development in the Neurobiology of Disease
    Jeffrey Noebels; Fiscal Year: 2006
    ....
  3. Parallel Sequence Profiling of Ion Channels in Epilepsy
    Jeffrey Noebels; Fiscal Year: 2007
    ..abstract_text> ..
  4. Excitability and Plasticity in Epileptic Brain
    Jeffrey Noebels; Fiscal Year: 2006
    ....
  5. Excitability and Plasticity in Developing Epileptic Brain
    Jeffrey Noebels; Fiscal Year: 2007
    ..Gene interactions that may prevent seizures from appearing are also explored, and may help predict the risk of childhood epilepsy. ..
  6. Excitability and Plasticity in Developing Epileptic Brain
    Jeffrey Noebels; Fiscal Year: 2009
    ..Gene interactions that may prevent seizures from appearing are also explored, and may help predict the risk of childhood epilepsy. ..
  7. PLASTICITY IN DEVELOPING EPILEPTIC BRAIN
    Jeffrey Noebels; Fiscal Year: 1993
    ....
  8. PLASTICITY IN DEVELOPING EPILEPTIC BRAIN
    Jeffrey 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. ..