Chapter category: Heat Shock Proteins
Assembly of Protein Aggregates in Neurodegeneration: Mechanisms Linking the Ubiquitin/Proteasome Pathway and Chaperones
Heat Shock Proteins in Neural Cells
Edited by: Christiane Richter-LandsbergISBN: 0-387-39952-6
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Chapter authors:
Sha-Ron Pierre, Vita Vernace, Zhiyou Wang and Maria E. Figueiredo-Pereira
In recent years, it has become increasingly evident that the majority of neurodegenerative disorders is associated with the aggregation and deposition of proteins in inclusion bodies. To avoid this abnormal deposition of proteins the cells recruit molecular chaperones to suppress aggregation and the ubiquitin/proteasome pathway (UPP) to remove the aggregate-prone proteins. The UPP is the major nonlysosomal degradation pathway for intracellular proteins. UPP impairment and/or its overload are likely to be major contributors to the aggregation of ubiquitinated proteins detected in most neuronal inclusion bodies. The mechanisms leading to the formation of inclusion bodies are not well defined. In this chapter, we discuss cellular strategies to deliver substrates to the UPP and their potential contribution to the development of intracellular proteins aggregates. In the future, a better understanding of the steps leading to protein aggregation and their deposition in inclusion bodies is likely to provide opportunities for effective therapeutic interventions.
Additional chapters from this book:
The Role of Heat Shock Proteins during Neurodegeneration in Alzheimer’s, Parkinson’s and Huntington’s Disease
Andreas Wyttenbach* and André Patrick Arrigo
A number of acute and chronic neurodegenerative conditions are associated by protein misfolding and aggregation of proteins within and outside cells. Misfolded proteins and protein aggregation are c...
Assembly of Protein Aggregates in Neurodegeneration: Mechanisms Linking the Ubiquitin/Proteasome Pathway and Chaperones
Sha-Ron Pierre, Vita Vernace, Zhiyou Wang and Maria E. Figueiredo-Pereira
In recent years, it has become increasingly evident that the majority of neurodegenerative disorders is associated with the aggregation and deposition of proteins in inclusion bodies. To avoid thi...
Heat Shock Proteins and the Regulation of Apoptosis
Una FitzGerald, Adrienne M. Gorman and Afshin Samali
Since the elucidation of their functions in protein folding and translocation, heat shock protein chaperones have been a target of research in all spheres of biomedicine. Within the last five years,...
The Role of Hsps in Neuronal Differentiation and Development
Kate Reed Herbert, Afshin Samali and Adrienne Gorman*
Heat shock proteins (Hsps) are expressed during development of the nervous system in a temporally and spatially controlled pattern that does not appear to be linked to activation of heat shock trans...
Heat Shock Proteins in Multiple Sclerosis
Celia F. Brosnan, Luca Battistini and Krzysztof Selmaj
In this review, we have addressed the possible contribution of heat shock proteins (HSP) to the pathogenesis of multiple sclerosis (MS), a chronic inflammatory demyelinating disease of the central ner...
Heme Oxygenase as a Therapeutic Funnel in Nutritional Redox Homeostasis and Cellular Stress Response: Role of Acetylcarnitine
Vittorio Calabrese, Giovanni Pennisi, Menotti Calvani, Annamaria Giuffrida, D. Allan Butterfield and Cesare Mancuso
Reduction of cellular expression and activity of antioxidant proteins and the consequent increase of oxidative stress are fundamental causes for both the aging processes and neurodegenerative diseases...
Heat Shock Proteins: Expression and Functional Roles in Nerve Cells and Glia
Christiane Richter-Landsberg
Heat shock proteins (HSPs) or so called stress proteins have multifunctional roles and are involved in many physiological processes, such as cell cycle control, cell proliferation, development, organi...
Small Heat Shock Proteins and the Cytoskeleton: Their Role in Inclusion Body Formation in Glial Cells
Christiane Richter-Landsberg and Olaf Goldbaum
The integrity of the cytoskeleton is an essential determinant for the function and survival of nerve cells and glia, and hence provides a sensitive target for stress situations. The small heat s...

