Prion DiseasesNeurodegenerative DiseasesPrionsAmyotrophic Lateral SclerosisPrPSc ProteinsPrPC ProteinsScrapieMenkes Kinky Hair SyndromeCreutzfeldt-Jakob SyndromeHuntington DiseaseSerine ProteasesSuperoxide DismutaseMice, TransgenicMotor NeuronsNerve DegenerationNeuronal Ceroid-LipofuscinosesLafora DiseaseDisease Models, AnimalBrainDipeptidyl-Peptidases and Tripeptidyl-PeptidasesEndopeptidase KEncephalopathy, Bovine SpongiformMutationNerve Tissue ProteinsProtein FoldingNeuronsSpinal CordSheepMice, Inbred C57BLAmino Acid SubstitutionAlzheimer DiseaseImmunohistochemistryBlotting, WesternMolecular Sequence DataParkinson Diseasealpha-Synucleintau ProteinsAmino Acid SequenceProtein ConformationHeredodegenerative Disorders, Nervous SystemTauopathiesNeuroprotective AgentsInclusion BodiesSynucleinsAmyloid beta-PeptidesSpinocerebellar AtaxiasMicrogliaTrinucleotide Repeat ExpansionProteostasis DeficienciesCell DeathOxidative StressFrontotemporal DementiaFrontotemporal Lobar DegenerationParkinsonian DisordersMitochondriaFriedreich AtaxiaLewy Body DiseaseAutophagyDementiaAmyloidAgingTrinucleotide RepeatsNeurotoxinsNeurofibrillary TanglesCells, CulturedLewy BodiesAstrocytesPeptidesHippocampusGuamSubstantia NigraAtaxiaModels, Biologicalbeta-SynucleinIntranuclear Inclusion BodiesCerebral CortexMultiple System AtrophySupranuclear Palsy, ProgressiveDopaminergic NeuronsNeurogliaMitochondrial DiseasesAxonal TransportCentral Nervous SystemUbiquitinPick Disease of the BrainMachado-Joseph DiseaseNuclear ProteinsProteasome Endopeptidase ComplexAtrophyAmyloid beta-Protein PrecursorCell SurvivalCentral Nervous System DiseasesSignal TransductionNervous System DiseasesIron-Binding ProteinsGliosisMotor Neuron DiseaseCorpus StriatumRotarod Performance TestGene Expression Regulation