2016
Reduced high-frequency motor neuron firing, EMG fractionation, and gait variability in awake walking ALS mice
Hadzipasic M, Ni W, Nagy M, Steenrod N, McGinley MJ, Kaushal A, Thomas E, McCormick DA, Horwich AL. Reduced high-frequency motor neuron firing, EMG fractionation, and gait variability in awake walking ALS mice. Proceedings Of The National Academy Of Sciences Of The United States Of America 2016, 113: e7600-e7609. PMID: 27821773, PMCID: PMC5127366, DOI: 10.1073/pnas.1616832113.Peer-Reviewed Original ResearchMeSH KeywordsAmyotrophic Lateral SclerosisAnimalsBiomechanical PhenomenaDisease Models, AnimalElectromyographyGaitHindlimbMiceMice, TransgenicMotor NeuronsMuscle, SkeletalMutationSuperoxide Dismutase-1ConceptsALS miceAmyotrophic lateral sclerosisAcute spinal cord slicesSingle-unit extracellular recordingsWhole-cell patch-clamp recordingsLoss of neuronsMotor neuron lossMotor neuron firingSpinal cord slicesPatch-clamp recordingsHigh-frequency firingStep variabilityLethal neurodegenerative diseaseNeuron lossCord slicesSpinal cordLeg flexorsLateral sclerosisGait variabilityVivo effectsClamp recordingsExtracellular recordingsEMG patternsMutant miceNeuron firing
2014
Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS
Hadzipasic M, Tahvildari B, Nagy M, Bian M, Horwich AL, McCormick DA. Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS. Proceedings Of The National Academy Of Sciences Of The United States Of America 2014, 111: 16883-16888. PMID: 25385594, PMCID: PMC4250117, DOI: 10.1073/pnas.1419497111.Peer-Reviewed Original ResearchMeSH KeywordsAmyotrophic Lateral SclerosisAnimalsMiceMotor NeuronsPatch-Clamp TechniquesSpinal CordSuperoxide DismutaseSuperoxide Dismutase-1ConceptsMN cell bodiesMotor neuronsMo of ageCell bodiesSpinal cordSpinal cord tissue slicesWhole-cell patch-clamp recordingsCell patch-clamp recordingsAdult mouse spinal cordIntrinsic electrophysiologic propertiesTwitch muscleSpinal motor neuronsLower extremity musclesTransgenic mouse modelMouse motor neuronsPatch-clamp recordingsAmyotrophic lateral sclerosisMouse spinal cordSlow-twitch muscleSteady-state firing ratePhysiological subtypesRetrograde tracingAcute slicesExtremity musclesPathophysiologic events
2013
RNA-Seq Profiling of Spinal Cord Motor Neurons from a Presymptomatic SOD1 ALS Mouse
Bandyopadhyay U, Cotney J, Nagy M, Oh S, Leng J, Mahajan M, Mane S, Fenton WA, Noonan JP, Horwich AL. RNA-Seq Profiling of Spinal Cord Motor Neurons from a Presymptomatic SOD1 ALS Mouse. PLOS ONE 2013, 8: e53575. PMID: 23301088, PMCID: PMC3536741, DOI: 10.1371/journal.pone.0053575.Peer-Reviewed Original ResearchConceptsRNA metabolismMutant proteinsMotor neuron cell bodiesPost-translational effectsRNA-seq profilingMotor neuronsNeuron cell bodiesWhole transcriptome profilingMonths of ageAltered expression levelsMutant human SOD1Transcriptome profilingLower motor neuron cell bodiesCell bodiesTransgenic strainsSpinal cord motor neuronsSymptoms of ALSMutant SOD1 miceSOD1-ALS miceAge-matched animalsHuman SOD1Amyotrophic lateral sclerosisProtein TDP43Expression levelsForms of ALS