Overview
- Includes supplementary material: sn.pub/extras
Part of the book series: Methods in Molecular Biology (MIMB, volume 232)
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About this book
For decades it has been known that structured conformations are important for the proper functioning of most cellular proteins. However, appreciation that protein folding to the functional conformations as well as the structural maintenance of protein molecules are very complex processes has only emerged during the last ten years. The intimate interplay uncovered by this scientific development led us to realize that perturbations of the protein folding process and disturbances of conformational maintenance are major disease mechanisms. This development has given rise to the concept of conformational diseases and the broader signature of protein folding diseases, comprising diseases in which mutations or environmental stresses may result in a partial misfolding that leads then to alternative conformations capable of disturbing cellular processes. This may happen by self-association (aggregation), as in prion and Alzheimer’s diseases, or by incorporation of alternatively folded subunits into structural entities, as in collagen diseases. Another possibility is that folding to the native structure is impaired or abolished, resulting in decreased stea- state levels of the correctly folded protein, as is observed in cystic fibrosis and 1-antitrypsin deficiency, as well as in many enzyme deficiencies. In addition, deficiencies of proteins that are engaged in assisting and supervising protein folding (protein quality control) may impair the folding of many other proteins, resulting in pathological phenotypes. Examples of this are the spastic paraplegia attributable to mutations in mitochondrial protease/chaperone complexes.
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Table of contents (24 protocols)
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Techniques in Conformational Disease Research
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Back Matter
Reviews
"...a very sound book that provides a solid background in conformational disease and presents the reader with helpful experimental protocols for studying protein folding folding and misfolding." - Pharmaceutical Research
"...this book will be a very useful took for specialists in protein chemistry, molecular biology and basic medicine, and also for teachers and their students studying principles of proteomics." Biochemistry
"...this book will be a very useful took for specialists in protein chemistry, molecular biology and basic medicine, and also for teachers and their students studying principles of proteomics." Biochemistry
Editors and Affiliations
Bibliographic Information
Book Title: Protein Misfolding and Disease
Editors: Peter Bross, Niels Gregersen
Series Title: Methods in Molecular Biology
DOI: https://doi.org/10.1385/1592593941
Publisher: Humana Totowa, NJ
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eBook Packages: Springer Book Archive
Copyright Information: Humana Press 2003
Hardcover ISBN: 978-1-58829-065-6Published: 20 June 2003
Softcover ISBN: 978-1-61737-332-9Published: 10 November 2010
eBook ISBN: 978-1-59259-394-1Published: 02 February 2008
Series ISSN: 1064-3745
Series E-ISSN: 1940-6029
Edition Number: 1
Number of Pages: XV, 318
Topics: Biochemistry, general