2023
Filamin C-associated Cardiomyopathy on Cardiac MR Images.
Weller S, Bartz-Overman C, Fuss C, Wu E. Filamin C-associated Cardiomyopathy on Cardiac MR Images. Radiology Cardiothoracic Imaging 2023, 5: e230165. PMID: 38166338, PMCID: PMC11163239, DOI: 10.1148/ryct.230165.Peer-Reviewed Original Research
2020
HtsRC-Mediated Accumulation of F-Actin Regulates Ring Canal Size During Drosophila melanogaster Oogenesis
Gerdes JA, Mannix KM, Hudson AM, Cooley L. HtsRC-Mediated Accumulation of F-Actin Regulates Ring Canal Size During Drosophila melanogaster Oogenesis. Genetics 2020, 216: 717-734. PMID: 32883702, PMCID: PMC7648574, DOI: 10.1534/genetics.120.303629.Peer-Reviewed Original ResearchConceptsGermline ring canalsRing canalsActin cytoskeletonF-actinDrosophila melanogaster oogenesisSomatic follicle cellsCombination of CRISPRF-actin accumulationF-actin recruitmentFilamentous actin cytoskeletonFemale germlineActin structuresFruit flyHigh fecundityFollicle cellsCytoskeletonGermlineOverexpressionAccumulationDrosophilaOogenesisMutagenesisCRISPRFilaminGenesFilamin 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
2019
Filamin A mediates isotropic distribution of applied force across the actin network
Kumar A, Shutova MS, Tanaka K, Iwamoto DV, Calderwood DA, Svitkina TM, Schwartz MA. Filamin A mediates isotropic distribution of applied force across the actin network. Journal Of Cell Biology 2019, 218: 2481-2491. PMID: 31315944, PMCID: PMC6683746, DOI: 10.1083/jcb.201901086.Peer-Reviewed Original ResearchConceptsTalin tension sensorStress fibersActin networkFilamin ACortical actin networkCortical actin filamentsIntegrin-mediated adhesionActin cytoskeletonFocal adhesionsCortical actinFLNA knockdownActin filamentsTalinKnockdownCell sensingDirection of stretchTension sensorPhysiology of muscleUniaxial stretchForce transmissionCytoskeletonStrainsStretchAdhesionReexpression
2018
Structural basis of the filamin A actin-binding domain interaction with F-actin
Iwamoto DV, Huehn A, Simon B, Huet-Calderwood C, Baldassarre M, Sindelar CV, Calderwood DA. Structural basis of the filamin A actin-binding domain interaction with F-actin. Nature Structural & Molecular Biology 2018, 25: 918-927. PMID: 30224736, PMCID: PMC6173970, DOI: 10.1038/s41594-018-0128-3.Peer-Reviewed Original ResearchConceptsActin-binding domainCalponin homology domainHomology domainF-actinActin cross-linking proteinFunction mutationsTandem calponin homology domainsDisease-associated mutantsCryo-electron microscopyHigh-resolution structuresNumerous genetic diseasesSequence conservationHigher-order structureLinking proteinStructural basisDomain interactionsCell shapeActin filamentsMolecular understandingN-terminalFunctional studiesGenetic diseasesMissense mutationsMutationsAtomic resolutionAn alternative splicing switch in FLNB promotes the mesenchymal cell state in human breast cancer
Li J, Choi P, Chaffer C, Labella K, Hwang J, Giacomelli A, Kim J, Ilic N, Doench J, Ly S, Dai C, Hagel K, Hong A, Gjoerup O, Goel S, Ge J, Root D, Zhao J, Brooks A, Weinberg R, Hahn W. An alternative splicing switch in FLNB promotes the mesenchymal cell state in human breast cancer. ELife 2018, 7: e37184. PMID: 30059005, PMCID: PMC6103745, DOI: 10.7554/elife.37184.Peer-Reviewed Original ResearchMeSH KeywordsAlternative SplicingAnimalsBase SequenceBreast NeoplasmsCell Line, TumorEpithelial-Mesenchymal TransitionExonsFemaleFilaminsGene Expression Regulation, NeoplasticGenome, HumanHumansHyaluronan ReceptorsMesenchymal Stem CellsMice, NudeNeoplasm ProteinsOpen Reading FramesProtein IsoformsReproducibility of ResultsRNA-Binding ProteinsRNA, MessengerConceptsEpithelial-to-mesenchymal transitionAlternative splicing of mRNA precursorsMesenchymal cell stateSplicing of mRNA precursorsCell statesRNA-binding proteinsAlternative splicing switchDysregulation of splicingBreast cancer patient samplesEMT gene signatureRegulation of epithelial-to-mesenchymal transitionCancer patient samplesInduce epithelial-to-mesenchymal transitionFOXC1 transcription factorRNA-seqAlternative splicingExpression screeningMRNA precursorsRegulating tumor cell plasticityRegulatory stepTranscription factorsSplicing switchProtein productionDiverse functionsIncreased tumorigenicity
2017
Enrichment of putatively damaging rare variants in the DYX2 locus and the reading-related genes CCDC136 and FLNC
Adams AK, Smith SD, Truong DT, Willcutt EG, Olson RK, DeFries JC, Pennington BF, Gruen JR. Enrichment of putatively damaging rare variants in the DYX2 locus and the reading-related genes CCDC136 and FLNC. Human Genetics 2017, 136: 1395-1405. PMID: 28866788, PMCID: PMC5702371, DOI: 10.1007/s00439-017-1838-z.Peer-Reviewed Original Research
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
2013
ASB2α, an E3 Ubiquitin Ligase Specificity Subunit, Regulates Cell Spreading and Triggers Proteasomal Degradation of Filamins by Targeting the Filamin Calponin Homology 1 Domain*
Razinia Z, Baldassarre M, Cantelli G, Calderwood DA. ASB2α, an E3 Ubiquitin Ligase Specificity Subunit, Regulates Cell Spreading and Triggers Proteasomal Degradation of Filamins by Targeting the Filamin Calponin Homology 1 Domain*. Journal Of Biological Chemistry 2013, 288: 32093-32105. PMID: 24052262, PMCID: PMC3814802, DOI: 10.1074/jbc.m113.496604.Peer-Reviewed Original ResearchConceptsHematopoietic cell differentiationSpecificity subunitProteasomal degradationF-actin-rich structuresE3 ubiquitin ligase complexCell differentiationNormal subcellular localizationHomology 1 domainLoss of filaminUbiquitin acceptor sitesActin-binding domainCross-linking proteinsActin-binding siteLigase complexActin cytoskeletonTransmembrane proteinSubcellular localizationΑ-actinin1Transient expressionASB2αDegradation of filaminMinimal fragmentLysine residuesFilaminCell adhesionMolecular characterization of the flightin gene in the wing-dimorphic planthopper, Nilaparvata lugens, and its evolution in Pancrustacea
Xue J, Zhang XQ, Xu HJ, Fan HW, Huang HJ, Ma XF, Wang CY, Chen JG, Cheng JA, Zhang CX. Molecular characterization of the flightin gene in the wing-dimorphic planthopper, Nilaparvata lugens, and its evolution in Pancrustacea. Insect Biochemistry And Molecular Biology 2013, 43: 433-443. PMID: 23459170, DOI: 10.1016/j.ibmb.2013.02.006.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsArthropod ProteinsBlotting, WesternCloning, MolecularDaphniaDrosophila melanogasterDrosophila ProteinsEvolution, MolecularFemaleFilaminsGene Expression RegulationHemipteraInsect ProteinsMaleMicroscopy, Electron, TransmissionMolecular Sequence DataMuscle ProteinsNymphPhylogenyPolymerase Chain ReactionRNA, Double-StrandedSequence AlignmentSex CharacteristicsConceptsBrown planthopperNilaparvata lugensMacropterous adultsDestructive rice pestsWing-dimorphic planthopperFlight muscle functionBrachypterous onesDorsal longitudinal musclesLong-distance migrationFemale brown planthopperDrosophila melanogasterArthropod speciesFlightin geneBPH adultsCrustacean speciesWing dimorphismRice pestsFlightinJuvenile instarsInsectsFunctional variationMolecular characterizationKey roleFlight apparatusSpecies
2012
Filamins in Mechanosensing and Signaling
Razinia Z, Mäkelä T, Ylänne J, Calderwood DA. Filamins in Mechanosensing and Signaling. Annual Review Of Biophysics 2012, 41: 227-246. PMID: 22404683, PMCID: PMC5508560, DOI: 10.1146/annurev-biophys-050511-102252.Peer-Reviewed Original ResearchConceptsPlasma membraneActin filamentsActin-binding proteinsExtracellular matrix connectionsCortical rigidityActin cytoskeletonCellular functionsCell cortexTranscription factorsTransmembrane receptorsAdhesion proteinsCell shapeFilaminIon channelsDiverse arrayFunctional evidenceEssential roleProteinMatrix connectionsPhysical forcesMembraneFilamentsCytoskeletalMechanosensingCytoskeletonMacrophage Mesenchymal Migration Requires Podosome Stabilization by Filamin A*
Guiet R, Vérollet C, Lamsoul I, Cougoule C, Poincloux R, Labrousse A, Calderwood DA, Glogauer M, Lutz PG, Maridonneau-Parini I. Macrophage Mesenchymal Migration Requires Podosome Stabilization by Filamin A*. Journal Of Biological Chemistry 2012, 287: 13051-13062. PMID: 22334688, PMCID: PMC3339984, DOI: 10.1074/jbc.m111.307124.Peer-Reviewed Original ResearchConceptsFilamin AMesenchymal migrationEmbryonic developmentPodosome rosette formationCell migrationMesenchymal migration modeCertain cell typesPodosome stabilityScaffold proteinActin polymerizationAmoeboid migrationNull mutationPodosomesActin filamentsMigratory cellsAmoeboid modeCell typesOrgan defectsMigration modesNew functionsThree-dimensional environmentMutationsProteaseStrong consequencesFLNA mutationsFilamin A controls matrix metalloproteinase activity and regulates cell invasion in human fibrosarcoma cells
Baldassarre M, Razinia Z, Brahme NN, Buccione R, Calderwood DA. Filamin A controls matrix metalloproteinase activity and regulates cell invasion in human fibrosarcoma cells. Journal Of Cell Science 2012, 125: 3858-3869. PMID: 22595522, PMCID: PMC3462082, DOI: 10.1242/jcs.104018.Peer-Reviewed Original ResearchMeSH KeywordsActinsCell AdhesionCell Line, TumorCell MovementContractile ProteinsEnzyme ActivationExtracellular MatrixFibrosarcomaFilaminsGene Knockdown TechniquesHumansIntegrinsMatrix Metalloproteinase 14Matrix Metalloproteinase 2Microfilament ProteinsNeoplasm InvasivenessPhenotypeProtein Structure, TertiaryConceptsFilamin AActin cytoskeletonCell invasionActin-binding domainCell surface adhesion proteinsControls cell motilityActin-binding proteinsIntegrin adhesion receptorsRandom cell migrationAbility of cellsArray of intracellularBreast cancer lossSurface adhesion proteinsHuman fibrosarcoma cellsExtracellular matrix degradationMatrix metalloproteinase activityFilamin expressionKnockdown cellsAdhesion proteinsCell motilityMetalloproteinase activityActin filamentsAdhesion receptorsFilaminECM remodeling
2011
The E3 ubiquitin ligase specificity subunit ASB2α targets filamins for proteasomal degradation by interacting with the filamin actin-binding domain
Razinia Z, Baldassarre M, Bouaouina M, Lamsoul I, Lutz PG, Calderwood DA. The E3 ubiquitin ligase specificity subunit ASB2α targets filamins for proteasomal degradation by interacting with the filamin actin-binding domain. Journal Of Cell Science 2011, 124: 2631-2641. PMID: 21750192, PMCID: PMC3138704, DOI: 10.1242/jcs.084343.Peer-Reviewed Original ResearchConceptsFilamin degradationProteasomal degradationCell differentiationDomain of filaminActin-rich structuresUbiquitin-proteasome pathwayExtracellular matrix connectionsActin cytoskeletonTransmembrane proteinSubcellular localizationMolecular basisSignaling cascadesASB2αActin filamentsFilaminAcute degradationBiochemical assaysMyeloid leukemia cellsImportant familyActinEarly eventsProteinLeukemia cellsImportant mechanismDifferentiation
2009
Filamins Regulate Cell Spreading and Initiation of Cell Migration
Baldassarre M, Razinia Z, Burande CF, Lamsoul I, Lutz PG, Calderwood DA. Filamins Regulate Cell Spreading and Initiation of Cell Migration. PLOS ONE 2009, 4: e7830. PMID: 19915675, PMCID: PMC2773003, DOI: 10.1371/journal.pone.0007830.Peer-Reviewed Original ResearchConceptsCell spreadingLarge actin-binding proteinCell biological analysesCell migrationActin-binding proteinsLoss of FlnAShRNA-mediated knockdownInitiation of migrationInhibition of initiationRecent knockout studiesProteasomal degradationKnockdown cellsInitiation of motilityKnockout studiesFilaminSingle knockoutImpairs migrationFLNAFLNBBiological analysisKnockdownProteinObserved defectsCellsPeriventricular heterotopiaFilamin A–β1 Integrin Complex Tunes Epithelial Cell Response to Matrix Tension
Gehler S, Baldassarre M, Lad Y, Leight JL, Wozniak MA, Riching KM, Eliceiri KW, Weaver VM, Calderwood DA, Keely PJ. Filamin A–β1 Integrin Complex Tunes Epithelial Cell Response to Matrix Tension. Molecular Biology Of The Cell 2009, 20: 3224-3238. PMID: 19458194, PMCID: PMC2710838, DOI: 10.1091/mbc.e08-12-1186.Peer-Reviewed Original ResearchConceptsFilamin AExtracellular matrixProtein filamin AHigh-density gelsMatrix tensionCollagen gelsMechanosensitive complexBreast epithelial cellsCellular contractilityMatrix stiffnessMorphogenesisEpithelial cell responsesCell typesDuctal morphogenesisEpithelial cellsCellsCollagen matrixGel contractionActinCollagen remodelingIntegrinsCell responsesCollagen fibrilsRemodelingGelThe E3 ubiquitin ligase specificity subunit ASB2β is a novel regulator of muscle differentiation that targets filamin B to proteasomal degradation
Bello NF, Lamsoul I, Heuzé ML, Métais A, Moreaux G, Calderwood DA, Duprez D, Moog-Lutz C, Lutz PG. The E3 ubiquitin ligase specificity subunit ASB2β is a novel regulator of muscle differentiation that targets filamin B to proteasomal degradation. Cell Death & Differentiation 2009, 16: 921-932. PMID: 19300455, PMCID: PMC2709956, DOI: 10.1038/cdd.2009.27.Peer-Reviewed Original ResearchMeSH KeywordsAdaptor Proteins, Signal TransducingAnimalsCarrier ProteinsCell DifferentiationCell LineChickensContractile ProteinsFilaminsGene Knockdown TechniquesHumansMiceMicrofilament ProteinsMyoblastsProteasome Endopeptidase ComplexRNA InterferenceRNA, MessengerSuppressor of Cytokine Signaling ProteinsUbiquitin-Protein LigasesConceptsFilamin BMuscle differentiationSpecificity subunitAnkyrin repeat-containing proteinActive E3 ubiquitin ligaseE3 ubiquitin ligase complexRepeat-containing proteinUbiquitin ligase complexE3 ubiquitin ligaseSuppressor of cytokineBox 2 geneLigase complexE3 ubiquitinUbiquitin ligaseProteasomal degradationMyoblast fusionNovel regulatorMuscle developmentKnockdown cellsProtein degradationMyogenic differentiationAdult tissuesC2C12 cellsMuscle contractile proteinsInduced differentiationThe Role of FilGAP-Filamin A Interactions in Mechanoprotection
Shifrin Y, Arora PD, Ohta Y, Calderwood DA, McCulloch CA. The Role of FilGAP-Filamin A Interactions in Mechanoprotection. Molecular Biology Of The Cell 2009, 20: 1269-1279. PMID: 19144823, PMCID: PMC2649276, DOI: 10.1091/mbc.e08-08-0872.Peer-Reviewed Original Research
2008
Structural Basis of the Migfilin-Filamin Interaction and Competition with Integrin β Tails*
Lad Y, Jiang P, Ruskamo S, Harburger DS, Ylänne J, Campbell ID, Calderwood DA. Structural Basis of the Migfilin-Filamin Interaction and Competition with Integrin β Tails*. Journal Of Biological Chemistry 2008, 283: 35154-35163. PMID: 18829455, PMCID: PMC2596399, DOI: 10.1074/jbc.m802592200.Peer-Reviewed Original ResearchConceptsCell-extracellular matrix adhesion sitesHuman filaminN-terminal actin-binding domainProtein-protein interaction studiesActin cross-linking protein filaminIntegrin β tailsMatrix adhesion sitesActin-binding domainIntegrin beta tailsN-terminal portionIntegrin-cytoskeleton linkagesImmunoglobulin-like domainsIntegrin tailsΒ tailAdaptor proteinMigfilinBeta tailsProtein filaminCommon binding siteMolecular basisStructural basisAdhesion sitesCell shapeFilaminCell adhesion
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