Jeffrey L. Noebels, M.D., Ph.D.

Professor of Neurology, Neuroscience, and
Molecular and Human Genetics
Director, Blue Bird Circle Developmental Neurogenetics Laboratory

Board Certification

American Board of Psychiatry and Neurology, Neurology

Medical School

M.D. (1981) Yale University

Graduate School

Ph.D. (1977) Stanford University

Residency

Neurology, Massachusetts General Hospital, Boston, MA

Clinical Interests

Epilepsy and Inherited Neurological Disease

Research Interests

The principal research strategy in the Developmental Neurogenetics Laboratory is to apply mutational analysis to learn how genes regulate neuronal excitability and network synchronization within the mammalian central nervous system. Spontaneous and transgenic mutations that express neurological phenotypes in the mouse provide a valuable opportunity to identify excitability genes and examine their role in synaptic plasticity in the developing brain. Brain wave (EEG) phenotypes emerge from altered neuronal signaling properties, and are of special interest. Six mouse mutants causing spike-wave synchronization of the neocortex have been discovered in our laboratory, and four (tottering, lethargic, ducky, and stargazer) are linked to mutations of subunits of neuronal voltage-gated calcium ion channels. Study of these mice have led to the identification of novel members of the gene family, and a new understanding of how related molecules rescue function and determine selective vulnerability within thalamocortical pathways. Other new mouse models for human epilepsy syndromes involving mutant ion channel, receptor, and synaptic vesicle proteins are being analyzed to pinpoint the neural network and specific electrophysiological abnormalities characteristic of the human disorder. We also explore the presynaptic release process and activity-induced changes of downstream gene expression in epileptic brain to identify regulatory pathways that are critical mechanisms of disease progression.

At present, mutant mouse models of inherited disorders in neuronal excitability are under investigation using in vitro cell physiology and optical fluorescence measurements of ion channel activity and exocytosis in presynaptic terminals of mouse brain slices, as well as molecular anatomical techniques including laser-capture microspcopy and realtime quantitative PCR, in situ hybridization and immunohistochemistry, and microarray analysis of seizure-activated mRNAs. These studies form the basis for development of strategies to selectively correct the tissue expression of neuronal gene errors early in development.

In collaboration with the Baylor Human Genome Sequencing Center, a large-scale translational genomic research study examining variants in human ion channel genes is currently in progress. The Human Channelopathy Project will evaluate the contribution of SNP profiles in several hundred ion channel subunit genes to the complex inheritance of neurological excitability disorders such as epilepsy.

Publications
Collapse All | Expand All

Ernst SJ, Aguilar-Bryan L, Noebels JL. Sodium channel beta1 regulatory subunit deficiency reduces pancreatic islet glucose-stimulated insulin and glucagon secretion. Endocrinology. 2009;150:1132-9.
Ernst WL, Noebels JL. Expanded alternative splice isoform profiling of the mouse Cav3.1/alpha1G T-type calcium channel. BMC Mol Biol. 2009;10:53.
Ernst WL, Zhang Y, Yoo JW, Ernst SJ, Noebels JL. Genetic enhancement of thalamocortical network activity by elevating alpha 1g-mediated low-voltage-activated calcium current induces pure absence epilepsy. J Neurosci. 2009;29:1615-25.
Jacobs MP, Leblanc GG, Brooks-Kayal A, Jensen FE, Lowenstein DH, Noebels JL, Spencer DD, Swann JW. Curing epilepsy: progress and future directions. Epilepsy Behav. 2009;14:438-45.
Glasscock E, Noebels JL. When a disease gene is not really a disease gene. Future Neurology. 2008;3:103-6.
Noebels JL. 2006 Merritt Putnam Symposium: mapping epileptic circuitry. Epilepsia. 2008;49 Suppl 3:1-2.
Seal RP, Akil O, Yi E, Weber CM, Grant L, Yoo J, Clause A, Kandler K, Noebels JL, Glowatzki E, Lustig LR, Edwards RH. Sensorineural deafness and seizures in mice lacking vesicular glutamate transporter 3. Neuron. 2008;57:263-75.
Shuba YM, Perez-Reyes E, Lory P, Noebels J. T-type calcium channels: from discovery to channelopathies, 25 years of research. Channels (Austin). 2008;2:299-302.
Singh NA, Otto JF, Dahle EJ, Pappas C, Leslie JD, Vilaythong A, Noebels JL, White HS, Wilcox KS, Leppert MF. Mouse models of human KCNQ2 and KCNQ3 mutations for benign familial neonatal convulsions show seizures and neuronal plasticity without synaptic reorganization. J Physiol. 2008;586:3405-23.
Bi W, Yan J, Shi X, Yuva-Paylor LA, Antalffy BA, Goldman A, Yoo JW, Noebels JL, Armstrong DL, Paylor R, Lupski JR. Rai1 deficiency in mice causes learning impairment and motor dysfunction, whereas Rai1 heterozygous mice display minimal behavioral phenotypes. Hum Mol Genet. 2007;16:1802-13.
Glasscock E, Qian J, Yoo JW, Noebels JL. Masking epilepsy by combining two epilepsy genes. Nat Neurosci. 2007;10:1554-8.
Lopez-Santiago LF, Meadows LS, Ernst SJ, Chen C, Malhotra JD, McEwen DP, Speelman A, Noebels JL, Maier SK, Lopatin AN, Isom LL. Sodium channel Scn1b null mice exhibit prolonged QT and RR intervals. J Mol Cell Cardiol. 2007;43:636-47.
Noebels JL, Sidman RL. Persistent hypersynchronization of neocortical neurons in the mocha mutant of mouse. J Neurogenet. 2007;21:253-6.
Palop JJ, Chin J, Roberson ED, Wang J, Thwin MT, Bien-Ly N, Yoo J, Ho KO, Yu GQ, Kreitzer A, Finkbeiner S, Noebels JL, Mucke L. Aberrant excitatory neuronal activity and compensatory remodeling of inhibitory hippocampal circuits in mouse models of Alzheimer's disease. Neuron. 2007;55:697-711.
Sisodiya S, Cross JH, Blumcke I, Chadwick D, Craig J, Crino PB, Debenham P, Delanty N, Elmslie F, Gardiner M, Golden J, Goldstein D, Greenberg DA, Guerrini R, Hanna M, Harris J, Harrison P, Johnson MR, Kirov G, Kullman DM, et al. Genetics of epilepsy: epilepsy research foundation workshop report. Epileptic Disord. 2007;9:194-236.
Goldman A, Noebels J. Invited comments on the Shostak and Ottman review. Epilepsia. 2006;47:1750-1; author reply 1755-6.
Goldman AM, Potocki L, Walz K, Lynch JK, Glaze DG, Lupski JR, Noebels JL. Epilepsy and chromosomal rearrangements in Smith-Magenis Syndrome [del(17)(p11.2p11.2)]. J Child Neurol. 2006;21:93-8.
Noebels JL. The Judith Hoyer Lecture: genes, pixels, patterns, and prevention. Epilepsy Behav. 2006;9:379-85.
Qian J, Noebels JL. Exocytosis of vesicular zinc reveals persistent depression of neurotransmitter release during metabotropic glutamate receptor long-term depression at the hippocampal CA3-CA1 synapse. J Neurosci. 2006;26:6089-95.
Wang D, Pascual JM, Yang H, Engelstad K, Mao X, Cheng J, Yoo J, Noebels JL, De Vivo DC. A mouse model for Glut-1 haploinsufficiency. Hum Mol Genet. 2006;15:1169-79.
Brenner R, Chen QH, Vilaythong A, Toney GM, Noebels JL, Aldrich RW. BK channel beta4 subunit reduces dentate gyrus excitability and protects against temporal lobe seizures. Nat Neurosci. 2005;8:1752-9.
Cobos I, Calcagnotto ME, Vilaythong AJ, Thwin MT, Noebels JL, Baraban SC, Rubenstein JL. Mice lacking Dlx1 show subtype-specific loss of interneurons, reduced inhibition and epilepsy. Nat Neurosci. 2005;8:1059-68.
Jankovic J, Noebels JL. Genetic mouse models of essential tremor: are they essential? J Clin Invest. 2005;115:584-6.
Noebels JL. Calcium channel 'gaiting' and absence epilepsy. Epilepsy Curr. 2005;5:95-7.
Qian J, Noebels JL. Visualization of transmitter release with zinc fluorescence detection at the mouse hippocampal mossy fibre synapse. J Physiol. 2005;566:747-58.
Senechal KR, Thaller C, Noebels JL. ADPEAF mutations reduce levels of secreted LGI1, a putative tumor suppressor protein linked to epilepsy. Hum Mol Genet. 2005;14:1613-20.
Chen C, Westenbroek RE, Xu X, Edwards CA, Sorenson DR, Chen Y, McEwen DP, O'Malley HA, Bharucha V, Meadows LS, Knudsen GA, Vilaythong A, Noebels JL, Saunders TL, Scheuer T, Shrager P, Catterall WA, Isom LL. Mice lacking sodium channel beta1 subunits display defects in neuronal excitability, sodium channel expression, and nodal architecture. J Neurosci. 2004;24:4030-42.
Collins AL, Levenson JM, Vilaythong AP, Richman R, Armstrong DL, Noebels JL, Sweatt JD, Zoghbi HY. Mild overexpression of MeCP2 causes a progressive neurological disorder in mice. Hum Mol Genet. 2004;13:2679-89.
May P, Rohlmann A, Bock HH, Zurhove K, Marth JD, Schomburg ED, Noebels JL, Beffert U, Sweatt JD, Weeber EJ, Herz J. Neuronal LRP1 functionally associates with postsynaptic proteins and is required for normal motor function in mice. Mol Cell Biol. 2004;24:8872-83.
Mori M, Burgess DL, Gefrides LA, Foreman PJ, Opferman JT, Korsmeyer SJ, Cavalheiro EA, Naffah-Mazzacoratti MG, Noebels JL. Expression of apoptosis inhibitor protein Mcl1 linked to neuroprotection in CNS neurons. Cell Death Differ. 2004;11:1223-33.
Zhang Y, Vilaythong AP, Yoshor D, Noebels JL. Elevated thalamic low-voltage-activated currents precede the onset of absence epilepsy in the SNAP25-deficient mouse mutant coloboma. J Neurosci. 2004;24:5239-48.
Houseweart MK, Pennacchio LA, Vilaythong A, Peters C, Noebels JL, Myers RM. Cathepsin B but not cathepsins L or S contributes to the pathogenesis of Unverricht-Lundborg progressive myoclonus epilepsy (EPM1). J Neurobiol. 2003;56:315-27.
Houseweart MK, Vilaythong A, Yin XM, Turk B, Noebels JL, Myers RM. Apoptosis caused by cathepsins does not require Bid signaling in an in vivo model of progressive myoclonus epilepsy (EPM1). Cell Death Differ. 2003;10:1329-35.
Kantheti P, Diaz ME, Peden AE, Seong EE, Dolan DF, Robinson MS, Noebels JL, Burmeister ML. Genetic and phenotypic analysis of the mouse mutant mh2J, an Ap3d allele caused by IAP element insertion. Mamm Genome. 2003;14:157-67.
Noebels JL. How a Sodium Channel Mutation Causes Epilepsy. Epilepsy Curr. 2003;3:70-71.
Noebels JL. Stiff Goats, Chloride Ions, and Idiopathic Generalized Epilepsy (IGE). Epilepsy Curr. 2003;3:146-147.
Noebels JL. The biology of epilepsy genes. Annu Rev Neurosci. 2003;26:599-625.
Noebels JL. Exploring new gene discoveries in idiopathic generalized epilepsy. Epilepsia. 2003;44 Suppl 2:16-21.
Powell EM, Campbell DB, Stanwood GD, Davis C, Noebels JL, Levitt P. Genetic disruption of cortical interneuron development causes region- and GABA cell type-specific deficits, epilepsy, and behavioral dysfunction. J Neurosci. 2003;23:622-31.
Qian J, Noebels JL. Topiramate alters excitatory synaptic transmission in mouse hippocampus. Epilepsy Res. 2003;55:225-33.
Walz K, Caratini-Rivera S, Bi W, Fonseca P, Mansouri DL, Lynch J, Vogel H, Noebels JL, Bradley A, Lupski JR. 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. Mol Cell Biol. 2003;23:3646-55.
Anderson E, Berkovic S, Dulac O, Gardiner M, Jain S, Laue Friis M, Lindhout D, Noebels J, Ottman R, Scaramelli A, Serratosa J, Steinlein O, Avanzini G, Bailey-Wilson J, Cardon L, Fischbach R, Gwinn-Hardy K, Leppert M, Ott J, Lindblad-Toh K, et al. ILAE genetics commission conference report: molecular analysis of complex genetic epilepsies. Epilepsia. 2002;43:1262-7.
Noebels JL. Human Epilepsy Can Be Linked to a Defective Calcium Channel. Epilepsy Curr. 2002;2:95.
Noebels JL. Sodium channel gene expression and epilepsy. Novartis Found Symp. 2002;241:109-20; discussion 120-3, 226-32.
Noebels JL. A Gene for JME at Last: The alpha1 GABA Receptor Subunit. Epilepsy Curr. 2002;2:131-132.
Poolos NP, Das S, Clark GD, Lardizabal D, Noebels JL, Wyllie E, Dobyns WB. Males with epilepsy, complete subcortical band heterotopia, and somatic mosaicism for DCX. Neurology. 2002;58:1559-62.
Shahbazian M, Young J, Yuva-Paylor L, Spencer C, Antalffy B, Noebels J, Armstrong D, Paylor R, Zoghbi H. Mice with truncated MeCP2 recapitulate many Rett syndrome features and display hyperacetylation of histone H3. Neuron. 2002;35:243-54.
Stables JP, Bertram EH, White HS, Coulter DA, Dichter MA, Jacobs MP, Loscher W, Lowenstein DH, Moshe SL, Noebels JL, Davis M. Models for epilepsy and epileptogenesis: report from the NIH workshop, Bethesda, Maryland. Epilepsia. 2002;43:1410-20.
Zhang Y, Mori M, Burgess DL, Noebels JL. Mutations in high-voltage-activated calcium channel genes stimulate low-voltage-activated currents in mouse thalamic relay neurons. J Neurosci. 2002;22:6362-71.
Burgess DL, Gefrides LA, Foreman PJ, Noebels JL. A cluster of three novel Ca2+ channel gamma subunit genes on chromosome 19q13.4: evolution and expression profile of the gamma subunit gene family. Genomics. 2001;71:339-50.
Heilstedt HA, Burgess DL, Anderson AE, Chedrawi A, Tharp B, Lee O, Kashork CD, Starkey DE, Wu YQ, Noebels JL, Shaffer LG, Shapira SK. Loss of the potassium channel beta-subunit gene, KCNAB2, is associated with epilepsy in patients with 1p36 deletion syndrome. Epilepsia. 2001;42:1103-11.
Jacobs MP, Fischbach GD, Davis MR, Dichter MA, Dingledine R, Lowenstein DH, Morrell MJ, Noebels JL, Rogawski MA, Spencer SS, Theodore WH. Future directions for epilepsy research. Neurology. 2001;57:1536-42.
Noebels JL. Modeling human epilepsies in mice. Epilepsia. 2001;42 Suppl 5:11-5.
Qian J, Noebels JL. Presynaptic Ca2+ channels and neurotransmitter release at the terminal of a mouse cortical neuron. J Neurosci. 2001;21:3721-8.
Qiao X, Suri C, Knusel B, Noebels JL. Absence of hippocampal mossy fiber sprouting in transgenic mice overexpressing brain-derived neurotrophic factor. J Neurosci Res. 2001;64:268-76.
Zwingman TA, Neumann PE, Noebels JL, Herrup K. Rocker is a new variant of the voltage-dependent calcium channel gene Cacna1a. J Neurosci. 2001;21:1169-78.
Ayata C, Shimizu-Sasamata M, Lo EH, Noebels JL, Moskowitz MA. Impaired neurotransmitter release and elevated threshold for cortical spreading depression in mice with mutations in the alpha1A subunit of P/Q type calcium channels. Neuroscience. 2000;95:639-45.
Burgess DL, Matsuura T, Ashizawa T, Noebels JL. Genetic localization of the Ca2+ channel gene CACNG2 near SCA10 on chromosome 22q13. Epilepsia. 2000;41:24-7.
Burgess DL, Noebels JL. Calcium channel defects in models of inherited generalized epilepsy. Epilepsia. 2000;41:1074-5.
Kellaway P, Mizrahi EM, Noebels JL. Benign focal epilepsies of childhood: genetically determined pathophysiology. Report of an international workshop. Epilepsia. 2000;41:1049-50.
Lau D, Vega-Saenz de Miera EC, Contreras D, Ozaita A, Harvey M, Chow A, Noebels JL, Paylor R, Morgan JI, Leonard CS, Rudy B. Impaired fast-spiking, suppressed cortical inhibition, and increased susceptibility to seizures in mice lacking Kv3.2 K+ channel proteins. J Neurosci. 2000;20:9071-85.
Liu M, Pleasure SJ, Collins AE, Noebels JL, Naya FJ, Tsai MJ, Lowenstein DH. Loss of BETA2/NeuroD leads to malformation of the dentate gyrus and epilepsy. Proc Natl Acad Sci U S A. 2000;97:865-70.
Matsuura T, Yamagata T, Burgess DL, Rasmussen A, Grewal RP, Watase K, Khajavi M, McCall AE, Davis CF, Zu L, Achari M, Pulst SM, Alonso E, Noebels JL, Nelson DL, Zoghbi HY, Ashizawa T. Large expansion of the ATTCT pentanucleotide repeat in spinocerebellar ataxia type 10. Nat Genet. 2000;26:191-4.
Qian J, Noebels JL. Presynaptic Ca(2+) influx at a mouse central synapse with Ca(2+) channel subunit mutations. J Neurosci. 2000;20:163-70.
Steinlein OK, Noebels JL. Ion channels and epilepsy in man and mouse. Curr Opin Genet Dev. 2000;10:286-91.
Burgess DL, Biddlecome GH, McDonough SI, Diaz ME, Zilinski CA, Bean BP, Campbell KP, Noebels JL. beta subunit reshuffling modifies N- and P/Q-type Ca2+ channel subunit compositions in lethargic mouse brain. Mol Cell Neurosci. 1999;13:293-311.
Burgess DL, Davis CF, Gefrides LA, Noebels JL. Identification of three novel Ca(2+) channel gamma subunit genes reveals molecular diversification by tandem and chromosome duplication. Genome Res. 1999;9:1204-13.
Burgess DL, Noebels JL. Voltage-dependent calcium channel mutations in neurological disease. Ann N Y Acad Sci. 1999;868:199-212.
Burgess DL, Noebels JL. Single gene defects in mice: the role of voltage-dependent calcium channels in absence models. Epilepsy Res. 1999;36:111-22.
Clark GD, Noebels JL. Cortin disaster: lissencephaly genes spell double trouble for the developing brain. Ann Neurol. 1999;45:141-2.
Hartmann HA, Colom LV, Sutherland ML, Noebels JL. Selective localization of cardiac SCN5A sodium channels in limbic regions of rat brain. Nat Neurosci. 1999;2:593-5.
Noebels JL. Single-gene models of epilepsy. Adv Neurol. 1999;79:227-38.
Sutherland ML, Williams SH, Abedi R, Overbeek PA, Pfaffinger PJ, Noebels JL. Overexpression of a Shaker-type potassium channel in mammalian central nervous system dysregulates native potassium channel gene expression. Proc Natl Acad Sci U S A. 1999;96:2451-5.
Jiang YH, Armstrong D, Albrecht U, Atkins CM, Noebels JL, Eichele G, Sweatt JD, Beaudet AL. Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation. Neuron. 1998;21:799-811.
Kantheti P, Qiao X, Diaz ME, Peden AA, Meyer GE, Carskadon SL, Kapfhamer D, Sufalko D, Robinson MS, Noebels JL, Burmeister M. Mutation in AP-3 delta in the mocha mouse links endosomal transport to storage deficiency in platelets, melanosomes, and synaptic vesicles. Neuron. 1998;21:111-22.
Nahm WK, Noebels JL. Nonobligate role of early or sustained expression of immediate-early gene proteins c-fos, c-jun, and Zif/268 in hippocampal mossy fiber sprouting. J Neurosci. 1998;18:9245-55.
Noebels JL. Ion Channelopathies and Heritable Epilepsy. News Physiol Sci. 1998;13:255-256.
Pennacchio LA, Bouley DM, Higgins KM, Scott MP, Noebels JL, Myers RM. Progressive ataxia, myoclonic epilepsy and cerebellar apoptosis in cystatin B-deficient mice. Nat Genet. 1998;20:251-8.
Westenbroek RE, Bausch SB, Lin RC, Franck JE, Noebels JL, Catterall WA. Upregulation of L-type Ca2+ channels in reactive astrocytes after brain injury, hypomyelination, and ischemia. J Neurosci. 1998;18:2321-34.
Burgess DL, Jones JM, Meisler MH, Noebels JL. Mutation of the Ca2+ channel beta subunit gene Cchb4 is associated with ataxia and seizures in the lethargic (lh) mouse. Cell. 1997;88:385-92.
Cattanach BM, Barr JA, Beechey CV, Martin J, Noebels J, Jones J. A candidate model for Angelman syndrome in the mouse. Mamm Genome. 1997;8:472-8.
Cox GA, Lutz CM, Yang CL, Biemesderfer D, Bronson RT, Fu A, Aronson PS, Noebels JL, Frankel WN. Sodium/hydrogen exchanger gene defect in slow-wave epilepsy mutant mice. Cell. 1997;91:139-48.
Di Pasquale E, Keegan KD, Noebels JL. Increased excitability and inward rectification in layer V cortical pyramidal neurons in the epileptic mutant mouse Stargazer. J Neurophysiol. 1997;77:621-31.
Kash SF, Johnson RS, Tecott LH, Noebels JL, Mayfield RD, Hanahan D, Baekkeskov S. Epilepsy in mice deficient in the 65-kDa isoform of glutamic acid decarboxylase. Proc Natl Acad Sci U S A. 1997;94:14060-5.
Noebels JL, Qiao X, Nahm WK. Neurogenetic approaches to axon sprouting in the hippocampus. Adv Neurol. 1997;72:35-44.
Noebels JL. Targeting epilepsy genes. Neuron. 1996;16:241-4.
Noebels JL, Sutherland ML, Nahm WK, DiPasquale E. Molecular and cellular plasticity in developing epileptic brain. Cold Spring Harb Symp Quant Biol. 1996;61:319-26.
Qiao X, Hefti F, Knusel B, Noebels JL. Selective failure of brain-derived neurotrophic factor mRNA expression in the cerebellum of stargazer, a mutant mouse with ataxia. J Neurosci. 1996;16:640-8.
Sutherland ML, Delaney TA, Noebels JL. Glutamate transporter mRNA expression in proliferative zones of the developing and adult murine CNS. J Neurosci. 1996;16:2191-207.
Chafetz RS, Nahm WK, Noebels JL. Aberrant expression of neuropeptide Y in hippocampal mossy fibers in the absence of local cell injury following the onset of spike-wave synchronization. Brain Res Mol Brain Res. 1995;31:111-21.
Noebels JL. Single locus mutations in mice expressing generalized spike-wave absence epilepsies. Ital J Neurol Sci. 1995;16:107-11.
Sutherland ML, Delaney TA, Noebels JL. Molecular characterization of a high-affinity mouse glutamate transporter. Gene. 1995;162:271-4.
Wang H, Allen ML, Grigg JJ, Noebels JL, Tempel BL. Hypomyelination alters K+ channel expression in mouse mutants shiverer and Trembler. Neuron. 1995;15:1337-47.
Frankel WN, Taylor BA, Noebels JL, Lutz CM. Genetic epilepsy model derived from common inbred mouse strains. Genetics. 1994;138:481-9.
Helekar SA, Noebels JL. Analysis of voltage-gated and synaptic conductances contributing to network excitability defects in the mutant mouse tottering. J Neurophysiol. 1994;71:1-10.
Buckwalter MS, Testa CM, Noebels JL, Camper SA. Genetic mapping and evaluation of candidate genes for spasmodic, a neurological mouse mutation with abnormal startle response. Genomics. 1993;17:279-86.
Qiao X, Noebels JL. Developmental analysis of hippocampal mossy fiber outgrowth in a mutant mouse with inherited spike-wave seizures. J Neurosci. 1993;13:4622-35.
Wu SM, Qiao X, Noebels JL, Yang XL. Localization and modulatory actions of zinc in vertebrate retina. Vision Res. 1993;33:2611-6.
Helekar SA, Noebels JL. A burst-dependent hippocampal excitability defect elicited by potassium at the developmental onset of spike-wave seizures in the Tottering mutant. Brain Res Dev Brain Res. 1992;65:205-10.
Westenbroek RE, Noebels JL, Catterall WA. Elevated expression of type II Na+ channels in hypomyelinated axons of shiverer mouse brain. J Neurosci. 1992;12:2259-67.
Helekar SA, Noebels JL. Synchronous hippocampal bursting reveals network excitability defects in an epilepsy gene mutation. Proc Natl Acad Sci U S A. 1991;88:4736-40.
Noebels JL. Mutational analysis of spike-wave epilepsy phenotypes. Epilepsy Res Suppl. 1991;4:201-12.
Noebels JL, Marcom PK, Jalilian-Tehrani MH. Sodium channel density in hypomyelinated brain increased by myelin basic protein gene deletion. Nature. 1991;352:431-4.
Qiao XX, Noebels JL. Genetic and phenotypic heterogeneity of inherited spike-wave epilepsy: two mutant gene loci with independent cerebral excitability defects. Brain Res. 1991;555:43-50.
Noebels JL, Qiao X, Bronson RT, Spencer C, Davisson MT. Stargazer: a new neurological mutant on chromosome 15 in the mouse with prolonged cortical seizures. Epilepsy Res. 1990;7:129-35.
Noebels JL, Rutecki PA. Altered hippocampal network excitability in the hypernoradrenergic mutant mouse tottering. Brain Res. 1990;524:225-30.
Noebels JL, Sidman RL. Persistent hypersynchronization of neocortical neurons in the mocha mutant of mouse. J Neurogenet. 1989;6:53-6.
Noebels JL. Mutational analysis of inherited epilepsies. Adv Neurol. 1986;44:97-113.
Noebels JL. A single gene error of noradrenergic axon growth synchronizes central neurones. Nature. 1984;310:409-11.
Noebels JL. Isolating single genes of the inherited epilepsies. Ann Neurol. 1984;16 Suppl:S18-21.
Levitt P, Noebels JL. Mutant mouse tottering: selective increase of locus ceruleus axons in a defined single-locus mutation. Proc Natl Acad Sci U S A. 1981;78:4630-4.
Noebels JL. Analysis of inherited epilepsy using single locus mutations in mice. Fed Proc. 1979;38:2405-10.
Noebels JL, Sidman RL. Inherited epilepsy: spike-wave and focal motor seizures in the mutant mouse tottering. Science. 1979;204:1334-6.
Noebels JL, Prince DA. Excitability changes in thalamocortical relay neurons during synchronous discharges in cat neocortex. J Neurophysiol. 1978;41:1282-96.
Noebels JL, Prince DA. Development of focal seizures in cerebral cortex: role of axon terminal bursting. J Neurophysiol. 1978;41:1267-81.
Noebels JL, Roth WT, Kopell BS. Cortical slow potentials and the occipital EEG in congenital blindness. J Neurol Sci. 1978;37:51-8.
Noebels JL, Pedley TA. Anatomic localization of topically applied [14C]penicillin during experimental focal epilepsy in cat neocortex. Brain Res. 1977;125:293-303.
Noebels JL, Prince DA. Presynaptic origin of penicillin after discharges at mammalian nerve terminals. Brain Res. 1977;138:59-74.
Schwartzkroin PA, Futamachi KJ, Noebels JL, Prince DA. Transcallosal effects of a cortical epileptiform focus. Brain Res. 1975;99:59-68.

Books and Volumes
Collapse All | Expand All

Kellaway P, Noebels JL. Problems and concepts in developmental neurophysiology. The Johns Hopkins series in contemporary medicine and public health. Baltimore: Johns Hopkins University Press; 1989.

Contact Information

Jeffrey L. Noebels, M.D., Ph.D.
Department of Neurology
Baylor College of Medicine
One Baylor Plaza, MS NB302
Houston, Texas 77030

Tel: 713-798-5860
Fax: 713-798-3771
Email: