Mechanism of neurodegenerative disease: role of the ubiquitin proteasome system
- 1 January 2004
- journal article
- review article
- Published by Taylor & Francis in Annals of Medicine
- Vol. 36 (4) , 315-320
- https://doi.org/10.1080/07853890410031948
Abstract
Many neurodegenerative disorders such as Alzheimer's disease (AD) Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD) are characterized by neuronal damage that may be caused by toxic, abnormal, aggregation‐prone proteins. The purpose of this review is threefold: 1) to provide the reader with an overview of the genes involved in the abnormal processing and accumulation of misfolded proteins in neurodegenerative diseases using PD as a model disease; 2) to understand the cellular mechanisms for disposal of abnormal proteins, and the effects of toxic protein accumulation on ubiquitin proteasome system (UPS) and neuronal survival and 3) to discuss the development and challenges of cell culture and animal models for a rational and effective treatment for these disorders.Keywords
This publication has 37 references indexed in Scilit:
- Rare genetic mutations shed light on the pathogenesis of Parkinson diseaseJournal of Clinical Investigation, 2003
- Parkin Protects against the Toxicity Associated with Mutant α-SynucleinNeuron, 2002
- The UCH-L1 Gene Encodes Two Opposing Enzymatic Activities that Affect α-Synuclein Degradation and Parkinson's Disease SusceptibilityCell, 2002
- Parkin functions as an E2-dependent ubiquitin– protein ligase and promotes the degradation of the synaptic vesicle-associated protein, CDCrel-1Proceedings of the National Academy of Sciences, 2000
- Synphilin-1 is present in Lewy bodies in Parkinson's diseaseAnnals of Neurology, 2000
- The ubiquitin pathway in Parkinson's diseaseNature, 1998
- Mutations in the parkin gene cause autosomal recessive juvenile parkinsonismNature, 1998
- Dopaminergic Neurons Intrinsic to the Primate StriatumJournal of Neuroscience, 1997
- Conformational diseaseThe Lancet, 1997
- The ubiquitin-proteasome proteolytic pathwayCell, 1994