The ubiquitin-proteasome system (UPS) has been implicated in neurodegenerative
diseases based on the presence of deposits consisting of ubiquitylated proteins in affected …

The post-translational attachment of one or several ubiquitin molecules to a protein
generates a variety of targeting signals that are used in many different ways in the cell …

Ataxin-3 is a ubiquitously expressed deubiqutinating enzyme with important functions in the
proteasomal protein degradation pathway and regulation of transcription. The C-terminus of …

The Ubiquitin-Proteasome Pathway (UPP), which is critical for normal function in the
nervous system and is implicated in various neurological diseases, requires the small …

The carboxy‐terminus of Hsc70 interacting protein (CHIP) is a cochaperone E3 ligase
containing three tandem repeats of tetratricopeptide (TPR) motifs and a C‐terminal U‐box …

Machado-Joseph disease or Spinocerebellar ataxia type 3 is a progressive fatal
neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3 …

Cerebellar ataxia (CA) is a disorder associated with impairments in balance, coordination,
and gait caused by degeneration of the cerebellum. The mutations associated with CA affect …

The cellular protein quality control system in association with aggresome formation
contributes to protecting cells against aggregation-prone protein-induced toxicity …

The proteasome is a cellular machine found in the cytosol, nucleus and on chromatin that
performs much of the proteolysis in eukaryotic cells. Recent reports show it is enriched at …

Abstract Ataxin-3 (AT3) is the protein that triggers the inherited neurodegenerative disorder
spinocerebellar ataxia type 3 when its polyglutamine (polyQ) stretch close to the C-terminus …