Heavy Chain DiseaseMetallochaperonesProteasome Endopeptidase ComplexUbiquitinCation Transport ProteinsMachado-Joseph DiseaseProteolysisAdenosine TriphosphatasesCopperHepatolenticular DegenerationHeredodegenerative Disorders, Nervous SystemMenkes Kinky Hair SyndromeNerve Tissue ProteinsProteinsNeurodegenerative DiseasesMolecular ChaperonesImmunoglobulin gamma-ChainsUbiquitin-Protein LigasesMolecular Sequence DataUbiquitinsUbiquitinationAmino Acid SequenceProtein BindingNuclear ProteinsPeptidesMutationCysteine EndopeptidasesCarrier ProteinsCell LineMuscle ProteinsMethylhistidinesMultienzyme ComplexesProtein Structure, TertiaryEndoplasmic Reticulum-Associated DegradationProteasome InhibitorsHuntington DiseaseProteostasis DeficienciesProtein TransportRepressor ProteinsPeptide HydrolasesLeupeptinsSKP Cullin F-Box Protein LigasesInclusion BodiesProtein FoldingSaccharomyces cerevisiaeSpinocerebellar AtaxiasAutophagyAmino Acid MotifsRecombinant Fusion ProteinsMuscular AtrophyModels, BiologicalCells, CulturedLeucineCysteine Proteinase InhibitorsF-Box ProteinsBiodegradation, EnvironmentalEndoplasmic ReticulumBlotting, WesternHeLa Cellsalpha-SynucleinSequence Homology, Amino AcidProtein Processing, Post-TranslationalCOS CellsTRPP Cation ChannelsBinding SitesTrinucleotide Repeat ExpansionEndopeptidase ClpEndopeptidasesLysosomesProtein StabilityProtease InhibitorsPhosphorylationModels, MolecularCytoplasmHydrolysisAmino AcidsBase SequenceUbiquitin-Conjugating EnzymesPolyubiquitinGolgi ApparatusSignal TransductionRecombinant ProteinsKineticsLigasesCell NucleusTransfectionUbiquitinated ProteinsDNA PrimersCloning, MolecularCullin ProteinsDNA-Binding ProteinsRNA, MessengerNeuronsATP-Dependent ProteasesSaccharomyces cerevisiae ProteinsProtein BiosynthesisMicroscopy, FluorescenceCalpainMice, TransgenicProtein Conformation