Research Topics
Genomes and Genes | Michael WongSummaryAffiliation: Washington University School of Medicine Country: USA Publications
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Publications
Cerebrospinal fluid protein concentration in pediatric patients: defining clinically relevant reference valuesM Wong
Department of Neurology, Washington University School of Medicine, St Louis, MO, USA
Arch Pediatr Adolesc Med 154:827-31. 2000....- Cerebrospinal fluid neuron-specific enolase following seizures in children: role of etiologyMichael Wong
Department of Neurology, Washington University School of Medicine and St Louis Children s Hospital, MO 63110 1093, USA
J Child Neurol 17:261-4. 2002..Children with cryptogenic, idiopathic, or febrile seizures, including status epilepticus, are at relatively low risk for neuronal damage following seizures... - An animal model of generalized nonconvulsive status epilepticus: immediate characteristics and long-term effectsMichael Wong
Department of Neurology and the Center for the Study of Nervous System Injury, Washington University School of Medicine, St Louis, MO 63110, USA
Exp Neurol 183:87-99. 2003..These results indicate that an animal model of GNCSE can be generated and that even in the absence of overt neuronal damage, GNCSE may produce functional changes in neurons that alter electrical excitability of neural circuits... 
Abnormal glutamate homeostasis and impaired synaptic plasticity and learning in a mouse model of tuberous sclerosis complexLing Hui Zeng
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
Neurobiol Dis 28:184-96. 2007..These results suggest that abnormal glutamate homeostasis predisposes to excitotoxic cell death, impaired synaptic plasticity and learning deficits in Tsc1 GFAP CKO mice...- Leptin inhibits 4-aminopyridine- and pentylenetetrazole-induced seizures and AMPAR-mediated synaptic transmission in rodentsLin Xu
Department of Neurology and Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, Missouri 63110, USA
J Clin Invest 118:272-80. 2008..Intranasal leptin administration may have potential as an acute abortive treatment for convulsive seizures in emergency situations... - Hippocampal seizures cause depolymerization of filamentous actin in neurons independent of acute morphological changesYannan Ouyang
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, MO 63110, USA
Brain Res 1143:238-46. 2007..In addition to affecting non-structural functions of spines, the degree to which overt structural changes occur with actin depolymerization is dependent on the severity and type of the pathophysiological stimulus... - Impaired astrocytic gap junction coupling and potassium buffering in a mouse model of tuberous sclerosis complexLin Xu
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
Neurobiol Dis 34:291-9. 2009..These findings suggest that Tsc1 inactivation in astrocytes causes defects in astrocytic gap junction coupling and potassium clearance, which may contribute to epilepsy in Tsc1(GFAP)CKO mice... - Modulation of astrocyte glutamate transporters decreases seizures in a mouse model of Tuberous Sclerosis ComplexLing Hui Zeng
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
Neurobiol Dis 37:764-71. 2010..These results indicate that astrocyte glutamate transporters contribute to epileptogenesis in Tsc1(GFAP)CKO mice and suggest novel therapeutic strategies for epilepsy in TSC directed at astrocytes... - The natural history and treatment of epilepsy in a murine model of tuberous sclerosisEbru Erbayat-Altay
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, U S A
Epilepsia 48:1470-6. 2007..Here, we characterize the natural history of the epilepsy in Tsc1(GFAP)CKO mice in more detail and report acute effects of treatment with standard antiepileptic drugs on seizures in these mice... - Mammalian target of rapamycin (mTOR) inhibition as a potential antiepileptogenic therapy: From tuberous sclerosis to common acquired epilepsiesMichael Wong
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
Epilepsia 51:27-36. 2010..Therefore, mTOR inhibition may represent a potential antiepileptogenic therapy for diverse types of epilepsy, including both genetic and acquired epilepsies... 
Increased severity of pentylenetetrazol induced seizures in leptin deficient ob/ob miceEbru Erbayat-Altay
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, United States
Neurosci Lett 433:82-6. 2008..These findings suggest that chronic leptin deficiency in vivo increases seizure susceptibility...- Brief seizures cause dendritic injuryDongjun Guo
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, MO 63110, USA
Neurobiol Dis 45:348-55. 2012..These studies demonstrate that seizures of all durations may trigger at least transient neuronal injury... - The mammalian target of rapamycin signaling pathway mediates epileptogenesis in a model of temporal lobe epilepsyLing Hui Zeng
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, Missouri 63110, USA
J Neurosci 29:6964-72. 2009..These findings indicate that mTOR signaling mediates mechanisms of epileptogenesis in the kainate rat model and that mTOR inhibitors have potential antiepileptogenic effects in this model... - Kainate seizures cause acute dendritic injury and actin depolymerization in vivoLing Hui Zeng
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, Missouri 63110, USA
J Neurosci 27:11604-13. 2007.... - Rapamycin prevents epilepsy in a mouse model of tuberous sclerosis complexLing Hui Zeng
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
Ann Neurol 63:444-53. 2008..Here, we tested whether rapamycin could prevent or reverse epilepsy, as well as other cellular and molecular brain abnormalities in Tsc1(GFAP)CKO mice... - Impaired glial glutamate transport in a mouse tuberous sclerosis epilepsy modelMichael Wong
Department of Neurology, Washington University School of Medicine, St Louis, MO, USA
Ann Neurol 54:251-6. 2003..These findings suggest that Tsc1 inactivation in astrocytes causes dysfunctional glutamate homeostasis, leading to seizure development in TSC... - The ketogenic diet inhibits the mammalian target of rapamycin (mTOR) pathwaySharon S McDaniel
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, Missouri 63110, USA
Epilepsia 52:e7-11. 2011..Because mTOR signaling has been implicated in epileptogenesis, these results suggest that the KD may have anticonvulsant or antiepileptogenic actions via mTOR pathway inhibition... - Animal models of focal cortical dysplasia and tuberous sclerosis complex: recent progress toward clinical applicationsMichael Wong
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, Missouri 63110, USA
Epilepsia 50:34-44. 2009.... - Pentylenetetrazole-induced seizures cause acute, but not chronic, mTOR pathway activation in ratBo Zhang
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, MO, USA
Epilepsia 53:506-11. 2012..To isolate the direct effects of seizures on the mTOR pathway, the time course and mechanisms of mTOR activation were investigated with acute seizures induced by pentylenetetrazole (PTZ), which does not lead to chronic epilepsy... - Transient decrease in F-actin may be necessary for translocation of proteins into dendritic spinesYannan Ouyang
Department of Neurology 8111, Washington University School of Medicine, 660 South Euclid, St Louis, MO 63110, USA
Eur J Neurosci 22:2995-3005. 2005.... - FGF14 regulates the intrinsic excitability of cerebellar Purkinje neuronsVikram G Shakkottai
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
Neurobiol Dis 33:81-8. 2009..6 expression in Purkinje neurons, and that the loss of FGF14 impairs spontaneous and repetitive firing in Purkinje neurons by altering the expression of Nav1.6 channels... - Tuberous sclerosis and epilepsy: role of astrocytesMichael Wong
Department of Neurology, Washington University School of Medicine, St Louis, Missouri, USA
Glia 60:1244-50. 2012..Targeting these defects in astrocytes may represent a novel therapeutic strategy for epilepsy in patients with TSC... - Video-EEG monitoring methods for characterizing rodent models of tuberous sclerosis and epilepsyNicholas R Rensing
Department of Neurology, The Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, MO, USA
Methods Mol Biol 821:373-91. 2012..These methodological approaches should be helpful in characterizing seizures and background EEG abnormalities not only in animal models of TSC but also in many rodent epilepsy models in general... - Mechanisms of epileptogenesis in tuberous sclerosis complex and related malformations of cortical development with abnormal glioneuronal proliferationMichael Wong
Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
Epilepsia 49:8-21. 2008..An improved understanding of these various factors involved in epileptogenesis has direct clinical implications for optimizing current treatments or developing novel therapeutic approaches for epilepsy in these disorders... - Epileptogenesis and reduced inward rectifier potassium current in tuberous sclerosis complex-1-deficient astrocytesLaura A Jansen
Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
Epilepsia 46:1871-80. 2005..Here, we investigated the hypothesis that impairment of potassium uptake through astrocyte inward rectifier potassium (Kir) channels may contribute to epileptogenesis in Tsc1(GFAP)CKO mice... - Rapamycin has paradoxical effects on S6 phosphorylation in rats with and without seizuresLinglin Chen
School of Medicine, Zhejiang University City College, Hangzhou, Zhejiang, China
Epilepsia 53:2026-33. 2012..In the present study, we examined this paradoxical effect of rapamycin in more detail, both in normal rats and KA-injected animals... - Astrocyte-specific TSC1 conditional knockout mice exhibit abnormal neuronal organization and seizuresErik J Uhlmann
Department of Neurology, Washington University School of Medicine, St Louis Children s Hospital, St Louis, MO 63110, USA
Ann Neurol 52:285-96. 2002..Collectively, our results demonstrate that astrocyte-specific disruption of Tsc1 in mice provides a context-dependent growth advantage for astrocytes that results in abnormalities in neuronal organization and epilepsy... - Leptin in cerebrospinal fluid from children: correlation with plasma leptin, sexual dimorphism, and lack of protein bindingM Landt
Department of Pediatrics, Washington University School of Medicine, St Louis Children s Hospital, MO 63110, USA
Clin Chem 46:854-8. 2000..Current evidence suggests that CSF leptin concentrations interact with hypothalamic centers controlling food intake. Serum leptin concentrations manifest a sexual dimorphism that arises during puberty... - Vigabatrin inhibits seizures and mTOR pathway activation in a mouse model of tuberous sclerosis complexBo Zhang
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, Missouri, United States of America
PLoS ONE 8:e57445. 2013..This study identifies a potential novel mechanism of action of an antiseizure medication involving the mTOR pathway, which may account for the unique efficacy of this drug for a genetic epilepsy... - Mammalian target of rapamycin (mTOR) inhibition: potential for antiseizure, antiepileptogenic, and epileptostatic therapyRobin C C Ryther
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, Box 8111, 660 South Euclid Avenue, St Louis, MO 63110, USA
Curr Neurol Neurosci Rep 12:410-8. 2012..Further studies are needed to assess the full potential of mTOR inhibitors for epilepsy treatment... - Therapeutic role of mammalian target of rapamycin (mTOR) inhibition in preventing epileptogenesisSharon S McDaniel
Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, MO 63110, USA
Neurosci Lett 497:231-9. 2011.... - In vivo imaging of dendritic spines during electrographic seizuresNicholas Rensing
Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, St. Louis, MO 63110, USA
Ann Neurol 58:888-98. 2005..Overall, our results suggest that most neocortical seizures have minimal acute effects on dendrites over several hours, but may predispose to dendritic injury under extreme conditions... - Acute intermittent porphyria: a diagnostic challengeElizabeth Anyaegbu
Department of Pediatrics, Division of Pediatric Nephrology, Washington University School of Medicine, St Louis, MO 63110, USA
J Child Neurol 27:917-21. 2012.... - Modulation of dendritic spines in epilepsy: cellular mechanisms and functional implicationsMichael Wong
Department of Neurology, Washington University School of Medicine, St Louis, MO, USA
Epilepsy Behav 7:569-77. 2005..Novel therapeutic strategies directed at modulation of spine motility may prevent the detrimental effects of seizures on cognitive function in epilepsy... - CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosisMegan A Griffey
Department of Internal Medicine, Washington University School of Medicine, St. Louis, MO 63110, USA
Mol Ther 13:538-47. 2006..However, higher levels or a broader distribution of PPT1 expression, or both, will be required for more complete correction of this neurodegenerative disease... - Analysis of cerebrospinal fluid glial fibrillary acidic protein after seizures in childrenChristina A Gurnett
Department of Neurology, Washington University School of Medicine, St. Louis Children's Hospital, Missouri 63110-1093, USA
Epilepsia 44:1455-8. 2003..Increased GFAP levels may occur in patients with normal NSE, suggesting that GFAP may be a more sensitive marker of brain injury in some cases... - Stabilizing dendritic structure as a novel therapeutic approach for epilepsyMichael Wong
Department of Neurology, Box 8111, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
Expert Rev Neurother 8:907-15. 2008..Mechanistically targeted approaches to limit seizure-related structural changes in dendrites may represent a novel therapeutic strategy for treating epilepsy and its complications... - Advances in the pathophysiology of developmental epilepsiesMichael Wong
Department of Neurology, Box 8111, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
Semin Pediatr Neurol 12:72-87. 2005.... - Ataxia and paroxysmal dyskinesia in mice lacking axonally transported FGF14Qing Wang
Department of Molecular Biology and Pharmacology, Washington University School of Medicine, St Louis 63110, USA
Neuron 35:25-38. 2002..Together, these studies identify a novel function for FGF14 in neuronal signaling and implicate FGF14 in axonal trafficking and synaptosomal function... - Postictal cerebrospinal fluid abnormalities in childrenM Wong
Department of Neurology, Washington University School of Medicine, St Louis, Missouri, USA
J Pediatr 138:373-7. 2001..To determine the frequency and characteristics of seizure-induced cerebrospinal fluid (CSF) abnormalities in children and to identify potential alternative causes of these findings... - Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosisCatherine Kielar
Pediatric Storage Disorders Laboratory, Box P040, MRC Social Genetic and Developmental Psychiatry Centre, Institute of Psychiatry, De Crespigny Park, King s College London, London, SE5 8AF, UK
Neurobiol Dis 25:150-62. 2007..These findings provide novel evidence for successive neuron loss within the thalamus and cortex in Ppt1-/- mice, revealing the thalamus as an important early focus of INCL pathogenesis... - The utility of tuberless models of tuberous sclerosisMichael Wong
Epilepsia 48:1629-30; author reply 1632-4. 2007 - The therapeutic efficacy of angiostatin against weakly- and highly-immunogenic 3LL tumorsMengfeng Li
Department of Pathology, University of Pittsburgh, University of Pittsburgh Cancer Institute, Pittsburgh, PA 15123, USA
In Vivo 16:577-82. 2002..Angiostatin treatment of immunized mice further enhanced the antitumor effect of tumor vaccination. CONCLUSION: Antitumor immune response could complement the therapeutic efficacy of angiostatin... - Fetal alcohol exposure impairs alveolar macrophage function via decreased glutathione availabilityTheresa W Gauthier
Department of Pediatrics, Emory University School of Medicine, 2040 Ridgewood Atlanta, GA 30322, USA
Pediatr Res 57:76-81. 2005..These results add a new dimension to the detrimental effects of fetal alcohol exposure on the developing alveolar macrophage, raising the possibility of GSH therapy to augment premature alveolar macrophage function...
Research Grants
- Rapid seizure-induced changes in dendritic spinesMichael Wong; Fiscal Year: 2007..Washington University possesses unmatched resources in cutting-edge technology and expert researchers in cellular imaging and represents an ideal environment for the PI to complete the goals of this proposal. ..
- Mechanisms of Brain Dysfunction in Tuberous SclerosisMichael Wong; Fiscal Year: 2007..Since epilepsy affects approximately 1% of all people, this research has the potential to have significant impact on public health. ..