2021
Age-dependent ataxia and neurodegeneration caused by an αII spectrin mutation with impaired regulation of its calpain sensitivity
Miazek A, Zalas M, Skrzymowska J, Bogin BA, Grzymajło K, Goszczynski TM, Levine ZA, Morrow JS, Stankewich MC. Age-dependent ataxia and neurodegeneration caused by an αII spectrin mutation with impaired regulation of its calpain sensitivity. Scientific Reports 2021, 11: 7312. PMID: 33790315, PMCID: PMC8012654, DOI: 10.1038/s41598-021-86470-1.Peer-Reviewed Original ResearchConceptsSpectrin cleavageCalpain cleavage sitesCalcium-activated proteaseGlobal neurodegenerationTraumatic encephalopathyC57BL/6J miceDendritic integrityExcessive activationNeuronal integrityProgressive ataxiaImpaired regulationCalpain activationCalpain sensitivityPhysiologic significanceNeurodegenerative diseasesNeuronal developmentCalpain proteolysisCalpain proteasesCalcium-dependent bindingAtaxiaNeurodegenerationCalpainActivated calpainSubstrate-level regulationCaM affinity
2014
A hierarchy of ankyrin-spectrin complexes clusters sodium channels at nodes of Ranvier
Ho TS, Zollinger DR, Chang KJ, Xu M, Cooper EC, Stankewich MC, Bennett V, Rasband MN. A hierarchy of ankyrin-spectrin complexes clusters sodium channels at nodes of Ranvier. Nature Neuroscience 2014, 17: 1664-1672. PMID: 25362473, PMCID: PMC4271271, DOI: 10.1038/nn.3859.Peer-Reviewed Original Research
2012
A Distal Axonal Cytoskeleton Forms an Intra-Axonal Boundary that Controls Axon Initial Segment Assembly
Galiano MR, Jha S, Ho TS, Zhang C, Ogawa Y, Chang KJ, Stankewich MC, Mohler PJ, Rasband MN. A Distal Axonal Cytoskeleton Forms an Intra-Axonal Boundary that Controls Axon Initial Segment Assembly. Cell 2012, 149: 1125-1139. PMID: 22632975, PMCID: PMC3361702, DOI: 10.1016/j.cell.2012.03.039.Peer-Reviewed Original Research
2011
Cell organization, growth, and neural and cardiac development require αII-spectrin
Stankewich MC, Cianci CD, Stabach PR, Ji L, Nath A, Morrow JS. Cell organization, growth, and neural and cardiac development require αII-spectrin. Journal Of Cell Science 2011, 124: 3956-3966. PMID: 22159418, PMCID: PMC3244980, DOI: 10.1242/jcs.080374.Peer-Reviewed Original ResearchMeSH KeywordsActinsAnimalsAnkyrinsAxonsBody PatterningCarrier ProteinsCell MembraneCell PolarityCell ProliferationCraniofacial AbnormalitiesEmbryo, MammalianEmbryonic DevelopmentFemaleFibroblastsGene DeletionHeart Defects, CongenitalMaleMiceMice, Inbred C57BLMicrofilament ProteinsNeural Tube DefectsNeuroepithelial CellsPhenotypeProtein StabilityPseudopodiaSpectrinConceptsΑII-spectrinSteady-state protein levelsΒIII spectrinEmbryonic day 12.5Tissue patterningRenal epithelial cellsEmbryonic lethalCortical actinOrgan developmentAnkyrin BExon trappingEmbryonic fibroblastsTranscriptional levelΒ-spectrinCardiac developmentCell organizationCell spreadingAxon formationNeural tubeHeterozygous animalsTargeted disruptionApical membraneNeuroepithelial cellsDay 12.5Cell morphology
2010
Targeted deletion of βIII spectrin impairs synaptogenesis and generates ataxic and seizure phenotypes
Stankewich MC, Gwynn B, Ardito T, Ji L, Kim J, Robledo RF, Lux SE, Peters LL, Morrow JS. Targeted deletion of βIII spectrin impairs synaptogenesis and generates ataxic and seizure phenotypes. Proceedings Of The National Academy Of Sciences Of The United States Of America 2010, 107: 6022-6027. PMID: 20231455, PMCID: PMC2851889, DOI: 10.1073/pnas.1001522107.Peer-Reviewed Original ResearchConceptsBetaIII spectrinSpinocerebellar ataxia type 5Spectrin membrane skeletonTargeted gene disruptionAmino acid transportersExcitatory amino acid transportersImpairs synaptogenesisProtein traffickingSeizure disorderGene disruptionDark Purkinje cellsMembrane skeletonAlphaII-spectrinAcid transportersMechanistic basisPurkinje cellsMembrane channelsGlutamate receptor deltaIntracellular pathwaysSpectrin bindsSynaptic proteinsSpectrinReceptor deltaProteinGolgi profiles