2019
Hsp110 mitigates α-synuclein pathology in vivo
Taguchi YV, Gorenberg EL, Nagy M, Thrasher D, Fenton WA, Volpicelli-Daley L, Horwich AL, Chandra SS. Hsp110 mitigates α-synuclein pathology in vivo. Proceedings Of The National Academy Of Sciences Of The United States Of America 2019, 116: 24310-24316. PMID: 31685606, PMCID: PMC6883785, DOI: 10.1073/pnas.1903268116.Peer-Reviewed Original ResearchConceptsΑ-synuclein pathologyOverexpression of Hsp110Α-synuclein aggregationPresynaptic protein α-synucleinΑ-synuclein seedsΑ-synuclein oligomersLewy bodiesMouse modelParkinson's diseaseCell culture modelSynaptic proteomeΑ-synucleinProtein α-synucleinPathologyCulture modelDiseaseMammalian cell culture modelsProtein changesOverexpressionVivoHsp110Molecular facilitatorsMiceUnbiased analysisBrain
2013
Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm
Song Y, Nagy M, Ni W, Tyagi NK, Fenton WA, López-Giráldez F, Overton JD, Horwich AL, Brady ST. Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm. Proceedings Of The National Academy Of Sciences Of The United States Of America 2013, 110: 5428-5433. PMID: 23509252, PMCID: PMC3619309, DOI: 10.1073/pnas.1303279110.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAxonal TransportBacterial ProteinsDecapodiformesGene Expression ProfilingHSP110 Heat-Shock ProteinsHumansLuminescent ProteinsMAP Kinase Kinase Kinase 5MiceMice, TransgenicMutation, MissenseP38 Mitogen-Activated Protein KinasesPhosphorylationProtein FoldingProteomicsRecombinant Fusion ProteinsSpinal CordSuperoxide DismutaseSuperoxide Dismutase-1Transport VesiclesConceptsMutant SOD1 proteinTransport defectSOD1 proteinApoptosis signal-regulating kinase 1 inhibitorsPhosphorylation of kinesinNucleotide exchange factorsMolecular chaperone Hsc70Different oligomeric statesYellow fluorescent proteinTransgenic miceProtein disaggregationChaperone Hsc70Exchange factorMAPK cascadePhosphorylation of p38Oligomeric stateDiminished phosphorylationKinase 1 inhibitorFusion proteinFluorescent proteinP38 inhibitorP38 MAPKPhosphorylationHsp110Cu/ZnRNA-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