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Protein Misfolding and Disease

Series: Methods in Molecular Biology

by Peter Bross and Niels Gregersen

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Main description:

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.

Contents:

I. General Chapters

Protein Misfolding, Aggregation, and Degradation in Disease
Niels Gregersen, Lars Bolund, and Peter Bross

Basic Introduction to In Vivo Protein Folding and Its Defects
Peter Bross and Niels Gregersen

Cystic Fibrosis: Premature Degradation of Mutant Proteins as a Molecular Disease Mechanism
Marina S. Gelman and Ron R. Kopito

a1-Antitrypsin Deficiency: Liver Disease Associated With Retention of a Mutant Secretory Glycoprotein in the Endoplasmic Reticulum, David H. Perlmutter

Parkinson's Disease: a-Synuclein and Parkin in Protein Aggregation and the Reversal of Unfolded Protein Stress
Lene Diness Jakobsen and Poul Henning Jensen

Aberrant Protein Folding as the Molecular Basis of Cancer
Melissa D. Scott and Judith Frydman

Yeast as a Model System to Investigate Protein Conformational Diseases
Christina M. Coughlan and Jeffrey L. Brodsky

II. General Methods

Expression of Recombinant Proteins: An Introduction
Jane Nohr, Karsten Kristiansen, and Anne-M. Krogsdam

Expression in E. coli Systems
Anne-M. Krogsdam, Karsten Kristiansen, and Jane Nohr

Protein Expression in Yeasts
Jane Nohr, Karsten Kristiansen, and Anne-M. Krogsdam

Site-Directed Mutagenesis
Jane Nohr and Karsten Kristiansen

Pulse-Chase Labeling Techniques for the Analysis of Protein Maturation and Degradation
Annemieke Jansens and Ineke Braakman

III. Techniques in Conformational Disease Research

Detection of Aggregates and Protein Inclusions by Staining of Tissues
James E. Galvin

Study of Mutant Proteins With Folding Defects in Cultured Patient Cells
Gabriele Dodt and Claudia Walter

E. coli Expression System for Identifying Folding Mutations of Human Adenosine Deaminase
Ines Santisteban, Francisco X. Arredondo-Vega, Shannon Daniels, andMichael S. Hershfield

Characterization of Overexpressed Mutant Proteins in Mammalian Cells
Thomas Juhl Corydon

Investigation of Unfolded-Protein Response in Cells Expressing Familial Alzheimer's Disease-Linked Presenilin Variants
Gopal Thinakaran, James W. Bowen, Daisuke Ito, Jae-Yoon Leem, Margaret L. Veselits, and Naoyuki Sato

Huntingtin Fragments Form Aggresome-Like Inclusion Bodies in Mammalian Cells
Annett Boeddrich, Rudi Lurz, and Erich E. Wanker

Application of Chemical Chaperones to the Rescue of Folding Defects
Tip W. Loo and David M. Clarke

Analysis of Defective Subunit Interactions Using the Two-Hybrid System
Belen Perez, Lourdes R. Desviat, and Magdalena Ugarte

Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in the Cytosol
Lourdes R. Desviat, Belen Perez, and Magdalena Ugarte

Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Microsomes
Alan W. Cuthbert and Will Fuller

Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Mitochondria
Peter Bross, Vibeke Winter, Christina Bak Pedersen, and Niels Gregersen

Investigation of Folding and Degradation of Mutant Proteins Synthesized in Semipermeabilized Cells
Cornelia M. Wilson and Neil J. Bulleid

Index

PRODUCT DETAILS

ISBN-13: 9781617373329
Publisher: Springer (Humana Press Inc.)
Publication date: November, 2010
Pages: 336
Weight: 495g
Availability: Available
Subcategories: Biochemistry

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