Amyotrophic Lateral SclerosisPrion DiseasesNeurodegenerative DiseasesPrionsPrPSc ProteinsScrapieSerine ProteasesDipeptidyl-Peptidases and Tripeptidyl-PeptidasesSuperoxide DismutaseMotor NeuronsMice, TransgenicDisease Models, AnimalBrainPrPC ProteinsMutationSheepHuntington DiseaseNerve DegenerationCreutzfeldt-Jakob SyndromeMenkes Kinky Hair SyndromeNeuronsNeuronal Ceroid-LipofuscinosesNerve Tissue ProteinsLafora DiseaseEndopeptidase KEncephalopathy, Bovine SpongiformProtein FoldingAlzheimer Diseasetau ProteinsParkinson Diseasealpha-SynucleinSpinal CordMice, Inbred C57BLTauopathiesNeuroprotective AgentsInclusion BodiesHeredodegenerative Disorders, Nervous SystemProteostasis DeficienciesMolecular Sequence DataMicrogliaImmunohistochemistryBlotting, WesternSynucleinsAmino Acid SequenceAmyloid beta-PeptidesAmino Acid SubstitutionSpinocerebellar AtaxiasCell DeathFrontotemporal Lobar DegenerationOxidative StressTrinucleotide Repeat ExpansionFrontotemporal DementiaMitochondriaProtein ConformationFriedreich AtaxiaAutophagyAgingAmyloidGuamCells, CulturedDementiaNeurofibrillary TanglesModels, BiologicalPeptidesAstrocytesLewy Body DiseaseNeurotoxinsParkinsonian DisordersLewy BodiesCentral Nervous SystemAxonal TransportTrinucleotide RepeatsMitochondrial DiseasesMotor Neuron DiseaseSupranuclear Palsy, ProgressiveHippocampusUbiquitinCentral Nervous System DiseasesSignal TransductionCerebral CortexIron-Binding ProteinsCycasProteasome Endopeptidase ComplexGene Expression RegulationMuscular Atrophy, SpinalCell SurvivalPick Disease of the BrainApoptosisNeurogliaRNA-Binding Protein FUSGliosisNervous System DiseasesNuclear ProteinsAnimals, Genetically ModifiedPlaque, AmyloidReactive Oxygen SpeciesPhenotypeMultiple System AtrophyMolecular ChaperonesSubstantia Nigra