Featured Publications
Filamin A inhibition reduces seizure activity in a mouse model of focal cortical malformations
Zhang L, Huang T, Teaw S, Nguyen LH, Hsieh LS, Gong X, Burns LH, Bordey A. Filamin A inhibition reduces seizure activity in a mouse model of focal cortical malformations. Science Translational Medicine 2020, 12 PMID: 32075941, DOI: 10.1126/scitranslmed.aay0289.Peer-Reviewed Original ResearchConceptsFocal cortical dysplasia type IITuberous sclerosis complexFocal cortical malformationsCortical malformationsSeizure frequencyReduced seizure frequencyVehicle-treated miceOnset of seizuresFilamin ALifelong epilepsyShort hairpin RNANeurological comorbiditiesNeuronal abnormalitiesSeizure activityEpilepsy treatmentSeizure onsetMouse modelAdult miceFLNA expressionPatientsCortical tissueMiceSeizuresMalformationsHairpin RNA
2024
Reducing Filamin A Restores Cortical Synaptic Connectivity and Early Social Communication Following Cellular Mosaicism in Autism Spectrum Disorder Pathways
Binder M, Escobar I, Xu Y, Sokolov A, Zhang L, Bordey A. Reducing Filamin A Restores Cortical Synaptic Connectivity and Early Social Communication Following Cellular Mosaicism in Autism Spectrum Disorder Pathways. Journal Of Neuroscience 2024, 44: e1245232024. PMID: 39164108, PMCID: PMC11426378, DOI: 10.1523/jneurosci.1245-23.2024.Peer-Reviewed Original ResearchPup isolation callsFilamin AIsolation callsAutism spectrum disorderSigns of autism spectrum disorderPyramidal neuronsConstitutively active mutantCommunication deficitsDownstream of ERK1/2MTOR complex 1Actin crosslinkingSynaptic connectionsExpression of RhebSpine densityActive mutantActive RhebCore pathwaysRhebCortical synaptic connectivityCellular mosaicismConstitutively active RhebOne-month-old miceExcitatory synaptic activityNeocortex of miceSocial communication
2016
Tsc1 haploinsufficiency is sufficient to increase dendritic patterning and Filamin A levels
Zhang L, Huang T, Bordey A. Tsc1 haploinsufficiency is sufficient to increase dendritic patterning and Filamin A levels. Neuroscience Letters 2016, 629: 15-18. PMID: 27345385, PMCID: PMC4983256, DOI: 10.1016/j.neulet.2016.06.037.Peer-Reviewed Original ResearchConceptsTuberous sclerosis complexDendritic complexityDendritic patterningTotal dendritic lengthTsc1 haploinsufficiencyFLNA levelsNeonatal electroporationDendritic lengthNewborn neuronsDendritic abnormalitiesSholl analysisOlfactory bulbFilamin ATsc1 lossHeterozygote miceCognitive defectsDendritic morphologyMiceA levelsMost individualsHaploinsufficiencyHeterozygote conditionLevelsAbnormalitiesNeurons
2014
MEK-ERK1/2-Dependent FLNA Overexpression Promotes Abnormal Dendritic Patterning in Tuberous Sclerosis Independent of mTOR
Zhang L, Bartley CM, Gong X, Hsieh LS, Lin TV, Feliciano DM, Bordey A. MEK-ERK1/2-Dependent FLNA Overexpression Promotes Abnormal Dendritic Patterning in Tuberous Sclerosis Independent of mTOR. Neuron 2014, 84: 78-91. PMID: 25277454, PMCID: PMC4185153, DOI: 10.1016/j.neuron.2014.09.009.Peer-Reviewed Original ResearchConceptsDendritic complexityFLNA overexpressionDendritic abnormalitiesFLNA expressionDendritic patterningComplex dendritic arborsWild-type neuronsFilamin ADendritic arborsERK1/2-dependent mannerDendritic defectsNeurological defectsMEK-ERK1/2NeuronsMTOR activityNeurodevelopmental disordersNeurodevelopmental diseasesProtein filamin AAbnormalitiesMTOROverexpression