title: Dysregulation of Ubiquitin-Proteasome System in Neurodegenerative Diseases.
abstract: The ubiquitin-proteasome system (UPS) is one of the major protein degradation pathways, where abnormal UPS function has been observed in cancer and neurological diseases. Many neurodegenerative diseases share a common pathological feature, namely intracellular ubiquitin-positive inclusions formed by aggregate-prone neurotoxic proteins. This suggests that dysfunction of the UPS in neurodegenerative diseases contributes to the accumulation of neurotoxic proteins and to instigate neurodegeneration. Here, we review recent findings describing various aspects of UPS dysregulation in neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, and Huntington's disease.
authors.affiliation: Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, College of Medicine, Collaborative Innovation Center for Brain Science, Xiamen University Xiamen, China.
authors.forename: Qiuyang
authors.initials: Q
authors.lastname: Zheng
authors.affiliation: Neuroscience Initiative, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, CA USA.
authors.forename: Timothy
authors.initials: T
authors.lastname: Huang
authors.affiliation: Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, College of Medicine, Collaborative Innovation Center for Brain Science, Xiamen University Xiamen, China.
authors.forename: Lishan
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, College of Medicine, Collaborative Innovation Center for Brain Science, Xiamen University Xiamen, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Zhou
authors.affiliation: Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, College of Medicine, Collaborative Innovation Center for Brain Science, Xiamen University Xiamen, China.
authors.forename: Hong
authors.initials: H
authors.lastname: Luo
authors.affiliation: Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, College of Medicine, Collaborative Innovation Center for Brain Science, Xiamen UniversityXiamen, China; Neuroscience Initiative, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, CAUSA.
authors.forename: Huaxi
authors.initials: H
authors.lastname: Xu
authors.affiliation: Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, College of Medicine, Collaborative Innovation Center for Brain Science, Xiamen University Xiamen, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Wang
keywords: Alzheimer’s disease; Huntington’s disease; Parkinson’s disease; deubiquitinating enzyme; proteasome; ubiquitin