Neurodegenerative diseases are severe disorders characterized by progressive neurodegeneration in specific brain regions. The
ubiquitin-proteasome system (UPS) is closely linked to neurodegenerative disease. In most cases, UPS impairment and dysregulation of
the UPS components are frequently observed. Moreover, toxin-induced neurodegeneration produces neuronal cell death accompanied by
decreased UPS function. These studies suggest an involvement of the UPS in these diseases. In this review, we summarize the changes to
UPS components in neurodegenerative diseases and the association between the UPS and disease pathology. Dysfunction of the UPS results
in the abnormal accumulation of proteins; thus, the UPS plays a critical role in disease pathogenesis. Drugs targeting specific components
of the UPS may provide promising strategies for disease treatment.
Keywords: Ubiquitin-proteasome system, deubiquitination, Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis.
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