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
2001
Calpain proteolysis of αII-spectrin in the normal adult human brain
Huh G, Glantz S, Je S, Morrow J, Kim J. Calpain proteolysis of αII-spectrin in the normal adult human brain. Neuroscience Letters 2001, 316: 41-44. PMID: 11720774, DOI: 10.1016/s0304-3940(01)02371-0.Peer-Reviewed Original ResearchConceptsAdult human brainCalpain activationEvident neurological diseaseHuman brainCortical gray matterLong-term potentiationNormal adult human brainDendrites of neuronsCerebellar Purkinje cellsCalpain processingReactive astrocytesCerebral cortexPyramidal neuronsSynaptic remodelingClinical conditionsNeuronal apoptosisAdult brainPositive cellsNeurological diseasesGray matterPurkinje cellsTopographic distributionAlphaII-spectrinBrainCalpain proteolysis
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