Robert Niescier
Associate Research ScientistAbout
Research
Publications
2025
All-optical visualization of specific molecules in the ultrastructural context of brain tissue
M’Saad O, Cairns A, Gulcicek J, Kasula R, Liao J, Kondratiuk I, Bewersdorf E, Kidd P, Falahati H, Gentile J, Niescier R, Watters K, Sterner R, Lee S, Guo X, Liu X, Desir G, De Camilli P, Rothman J, Koleske A, Biederer T, Kuan A, Bewersdorf J. All-optical visualization of specific molecules in the ultrastructural context of brain tissue. Nature Biotechnology 2025, 1-15. PMID: 41299044, DOI: 10.1038/s41587-025-02905-4.Peer-Reviewed Original ResearchUltrastructural contextCellular organellesMolecular anatomyCell-specific antibodiesPostsynaptic densityAnatomical ultrastructureThree-dimensional contextNeuronal culturesImage volumesNeuronal circuitryProteinNanoscale distributionUltrastructureBrain tissue sectionsMolecular specificityTissue sectionsBrain ultrastructureSpecific moleculesBrain tissueOrganellesNeural connectionsA CRISPR activation screen reveals a cilia disassembly pathway mutated in focal cortical dysplasia
Elliott S, Ready P, Wrinn C, Ma Q, Edward M, Niescier R, Escobar I, Sun J, Ganga A, McAtee C, Atiş İ, Koleske A, Bordey A, Breslow D. A CRISPR activation screen reveals a cilia disassembly pathway mutated in focal cortical dysplasia. Science Advances 2025, 11: eaeb7238. PMID: 41160700, PMCID: PMC12571082, DOI: 10.1126/sciadv.aeb7238.Peer-Reviewed Original ResearchConceptsCilia disassemblyCRISPR activationNegative regulatorAssembly of primary ciliaDefective assemblyFocal cortical dysplasiaGenome-wide CRISPR activationAberrant pathway activationCRISPR activation screenSARM1 inhibitionG protein-coupled receptorsCortical dysplasiaTIR motif-containing 1RHOA mutationsPrimary ciliaFunctional pathwaysRhoASomatic mutationsDisassembly pathwayCiliary functionSterile alphaPathway activationFunctional impactMutationsCilia
2022
Atypical nuclear envelope condensates linked to neurological disorders reveal nucleoporin-directed chaperone activities
Prophet SM, Rampello AJ, Niescier RF, Gentile JE, Mallik S, Koleske AJ, Schlieker C. Atypical nuclear envelope condensates linked to neurological disorders reveal nucleoporin-directed chaperone activities. Nature Cell Biology 2022, 24: 1630-1641. PMID: 36302970, PMCID: PMC10041656, DOI: 10.1038/s41556-022-01001-y.Peer-Reviewed Original Research
2021
The Potential Role of AMPA Receptor Trafficking in Autism and Other Neurodevelopmental Conditions
Niescier R, Lin Y. The Potential Role of AMPA Receptor Trafficking in Autism and Other Neurodevelopmental Conditions. Neuroscience 2021, 479: 180-191. PMID: 34571086, DOI: 10.1016/j.neuroscience.2021.09.013.Peer-Reviewed Original ResearchConceptsLong-term potentiationTrafficking of AMPA receptorsLow-penetrance genesAMPA receptor traffickingPost-synaptic surfaceTuning of neuronal connectivityIonotropic glutamate receptorsPost-synaptic areasAutism spectrum disorderAMPA receptorsGlutamate receptorsSurface traffickingTuberous sclerosisExcitatory connectionsNeurodevelopmental conditionsReceptor traffickingGenetic heterogeneityNeuronal connectivityRett syndromePost-synapseFragile X syndromeMolecular basisGenesReceptorsTraffickingRegulation of Neural Circuit Development by Cadherin-11 Provides Implications for Autism
Frei J, Niescier R, Bridi M, Durens M, Nestor J, Kilander M, Yuan X, Dykxhoorn D, Nestor M, Huang S, Blatt G, Lin Y. Regulation of Neural Circuit Development by Cadherin-11 Provides Implications for Autism. ENeuro 2021, 8: eneuro.0066-21.2021. PMID: 34135003, PMCID: PMC8266214, DOI: 10.1523/eneuro.0066-21.2021.Peer-Reviewed Original ResearchConceptsNeural precursor cellsAutism spectrum disorderEtiology of autismCadherin-8Development of neuronal circuitryCadherin-11Postsynaptic density (PSD)-95Expression of cadherin-8Expression levelsNeuroligin-1Cortical neural precursor cellsKnock-outPluripotent stem cellsNeural circuit developmentFunction of cadherin-11KO brainHuman tissuesMouse brain developmentSynaptic activityNeuronal circuitryDendritic complexityRepetitive behaviorsExpression levels of cadherin-11Precursor cellsStem cells
2020
Development of a 3-D Organoid System Using Human Induced Pluripotent Stem Cells to Model Idiopathic Autism
Lunden J, Durens M, Nestor J, Niescier R, Herold K, Brandenburg C, Lin Y, Blatt G, Nestor M. Development of a 3-D Organoid System Using Human Induced Pluripotent Stem Cells to Model Idiopathic Autism. Advances In Neurobiology 2020, 25: 259-297. PMID: 32578151, DOI: 10.1007/978-3-030-45493-7_10.Peer-Reviewed Original ResearchConceptsGenetic diversityImpact of gene-environment interactionsGenetic backgroundAutism susceptibility genesHeterogeneous genetic backgroundHigh-content assayYamanaka transcription factorsGene-environment interactionsTranscription factorsDevelopment of therapeuticsSusceptibility genesTest cohortCell typesPhenotypic studiesPeripheral blood mononuclear cellsDental pulp cellsBlood mononuclear cellsTherapeutic developmentAutism spectrum conditionsRenewing cell typesInduction of iPSCsNeurophysiological aspectsPulp cellsIdiopathic autismTreatment optionsTomosyn regulates the small RhoA GTPase to control the dendritic stability of neurons and the surface expression of AMPA receptors
Shen W, Kilander M, Bridi M, Frei J, Niescier R, Huang S, Lin Y. Tomosyn regulates the small RhoA GTPase to control the dendritic stability of neurons and the surface expression of AMPA receptors. Journal Of Neuroscience Research 2020, 98: 1213-1231. PMID: 32133675, PMCID: PMC7216846, DOI: 10.1002/jnr.24608.Peer-Reviewed Original ResearchConceptsSurface expression of AMPA receptorsExpression of AMPA receptorsSoluble N-ethylmaleimide-sensitive factor attachment protein receptor machineryLoss-of-function phenotypesTrafficking of ionotropic glutamate receptorsFunction of tomosynRhoA GTPase activitySingle nucleotide mutationsPostsynaptic roleMouse primary neuronsDendrite stabilizationAMPA receptorsSurface expressionWD40 motifGTPase activityRhoA GTPaseShort hairpin RNATomosynRhoA signalingRhoA activationNucleotide mutationsCellular functionsLoss of dendritic spinesRhoACanonical interactionsHigh-throughput screening of human induced pluripotent stem cell-derived brain organoids
Durens M, Nestor J, Williams M, Herold K, Niescier R, Lunden J, Phillips A, Lin Y, Dykxhoorn D, Nestor M. High-throughput screening of human induced pluripotent stem cell-derived brain organoids. Journal Of Neuroscience Methods 2020, 335: 108627. PMID: 32032714, DOI: 10.1016/j.jneumeth.2020.108627.Peer-Reviewed Original ResearchConceptsSerum-free embryoid bodiesMulti-electrode arrayMulti-electrode array analysisSingle-cell calcium imagingHigh-content imagingBlockade of NMDARsHigh-throughput screeningStem cell platformsPhenotypic drug screeningExcitatory neuronsCalcium imagingProcessing speedCortical organoidsCellular compositionExperimental replicatesEmbryoid bodiesUnbiased analysisEfficient starting pointBlockadeOrganoidsComplex assaysDisease modelsPlatformDrug screeningNeurite outgrowth
2018
MCU Interacts with Miro1 to Modulate Mitochondrial Functions in Neurons
Niescier R, Hong K, Park D, Min K. MCU Interacts with Miro1 to Modulate Mitochondrial Functions in Neurons. Journal Of Neuroscience 2018, 38: 4666-4677. PMID: 29686046, PMCID: PMC6596018, DOI: 10.1523/jneurosci.0504-18.2018.Peer-Reviewed Original ResearchConceptsMitochondrial calcium uniporterMitochondrial movementMitochondrial transportMitochondrial CaMembrane proteinsN-terminusRegulation of mitochondrial transportOuter mitochondrial membrane proteinMitochondrial calcium uniporter complexMitochondrial functionMitochondrial Ca2+ uptakeMitochondrial calciumMitochondrial calcium uniporter (MCU) complexMitochondrial targeting sequenceMitochondrial membrane proteinOuter mitochondrial membraneN-terminal domainMitochondrial calcium levelsMitochondrial metabolic activityMitochondrial targetingMitochondrial membraneMiro1Target sequenceTransmembrane domainCalcium uniporter
2016
Dynamics of Mitochondrial Transport in Axons
Niescier R, Kwak S, Joo H, Chang K, Min K. Dynamics of Mitochondrial Transport in Axons. Frontiers In Cellular Neuroscience 2016, 10: 123. PMID: 27242435, PMCID: PMC4865487, DOI: 10.3389/fncel.2016.00123.Peer-Reviewed Original Research