There is increasing evidence that links together various common neurodegenerative diseases, such as Parkinson's and Alzheimer's diseases. Finding common themes in the pathophysiology of such disorders is increasingly important. This text addresses such a common theme-the proteasome. Since the discovery of the proteasome some 20 years ago, significant strides have been made in our understanding of proteasome biology and our understanding of the role the proteasome plays in a wide variety of biochemical events. Taken together, these studies have confirmed an important role for the proteasome in the fields of cell biology, oncology, immunology, gerontology, and neuroscience. The focus of this book is to provide an in depth analysis and provocative discussion of what role normal and aberrant proteasome function plays in regulating various aspects of brain homeostasis. TOC:Yeast Proteasome Structure and Biogenesis.- Ubiquitin-Proteasome Function in the Central Nervous System.- Protein Aggregation and the UPS: A Two Way Street.- Impact of Inclusion Formation on Cell Survival.- Inclusion Formation and Dissolution Following Proteasomal Inhibition in Neuronal Cells.- The Proteasome: Both A Source and A Target of Oxidative Stress?.- Inflammation as A Mediator of Oxidative Stress and UPS Dysfunction.- Role of The Ubiquitin Proteasome System During Neuronal Cell Death.- Mechanisms of Neuronal Death Induced by Proteasomal Inhibition.- Pharmacological And Molecular Models of Proteasomal Dysfunction.- The Gad Mouse: A Window into UPS-Related Neurodegeneration and the Function of The Deubiquitinating Enzyme UCH-L1.- Parkinson's Disease and Related Disorders.- Ubiquitination by Parkin-Implications in Parkinson's Disease.- Huntington's Disease.- Frameshift Ubiquitin Mutant in Alzheimer's Disease and Other Neurodegenerative Disorders .- Motor Neuron Disease.- The Paradoxical Role of Proteasomes in Prion Diseases.- Aging and The Proteasome.
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