1. Protein Misfolding, Aggregation, and Degradation in Disease
Niels Gregersen; Lars Bolund; Peter Bross
pp. 3-16
2. Basic Introduction to In Vivo Protein Folding and Its Defects
Peter Bross; Niels Gregersen
pp. 17-26
3. Cystic Fibrosis: Premature Degradation of Mutant Proteins as a Molecular Disease Mechanism
Marina Gelman; Ron Kopito
pp. 27-38
4. ?1-Antitrypsin Deficiency: Liver Disease Associated With Retention of a Mutant Secretory Glycoprotein in the Endoplasmic Reticulum
David Perlmutter
pp. 39-56
5. Parkinson's Disease: ? -Synuclein and Parkin in Protein Aggregation and the Reversal of Unfolded Protein Stress
Lene Jakobsen; Poul Jensen
pp. 57-66
6. Aberrant Protein Folding as the Molecular Basis of Cancer
Melissa Scott; Judith Frydman
pp. 67-76
7. Yeast as a Model System to Investigate Protein Conformational Diseases
Christina Coughlan; Jeffrey Brodsky
pp. 77-90
8. Expression of Recombinant Proteins: An Introduction
Jane Nøhr; Karsten Kristiansen; Anne-M. Krogsdam
pp. 93-102
9. Expression in E. coli Systems
Anne-M. Krogsdam; Karsten Kristiansen; Jane Nøhr
pp. 103-110
10. Protein Expression in Yeasts
Jane Nøhr; Karsten Kristiansen; Anne-M. Krogsdam
pp. 111-126
11. Site-Directed Mutagenesis
Jane Nøhr; Karsten Kristiansen
pp. 127-132
12. Pulse-Chase Labeling Techniques for the Analysis of Protein Maturation and Degradation
Annemieke Jansens; Ineke Braakman
pp. 133-146
13. Detection of Aggregates and Protein Inclusions by Staining of Tissues
James Galvin
pp. 149-164
14. Study of Mutant Proteins With Folding Defects in Cultured Patient Cells
Gabriele Dodt; Claudia Walter
pp. 165-174
15. E. coli Expression System for Identifying Folding Mutations of Human Adenosine Deaminase
Ines Santisteban; Francisco Arredondo-Vega; Shannon Daniels; Michael Hershfield
pp. 175-182
16. Characterization of Overexpressed Mutant Proteins in Mammalian Cells
Thomas Corydon
pp. 183-202
17. Investigation of Unfolded-Protein Response in Cells Expressing Familial Alzheimer's Disease-Linked Presenilin Variants
Gopal Thinakaran; James Bowen; Daisuke Ito; Jae-Yoon Leem; Margaret Veselits; Naoyuki Sato
pp. 203-216
18. Huntingtin Fragments Form Aggresome-Like Inclusion Bodies in Mammalian Cells
Annett Boeddrich; Rudi Lurz; Erich Wanker
pp. 217-230
19. Application of Chemical Chaperones to the Rescue of Folding Defects
Tip Loo; David Clarke
pp. 231-244
20. Analysis of Defective Subunit Interactions Using the Two-Hybrid System
Belén Pérez; Lourdes Desviat; Magdalena Ugarte
pp. 245-256
21. Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in the Cytosol
Lourdes Desviat; Belén Pérez; Magdalena Ugarte
pp. 257-264
22. Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Microsomes
Alan Cuthbert; Will Fuller
pp. 265-284
23. Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Mitochondria
Peter Bross; Vibeke Winter; Christina Pedersen; Niels Gregersen
pp. 285-294
24. Investigation of Folding and Degradation of Mutant Proteins Synthesized in Semipermeabilized Cells
Cornelia Wilson; Neil Bulleid
pp. 295-312
Abstract:
A comprehensive review of the latest thinking about the molecular processes underlying conformational diseases, combined with a remarkable set of biochemical, genomic cellular, and chemical laboratory techniques for studying their genesis and pathologies. The authors apply their carefully refined methods to a variety of metabolic and neurodegenerative disorders, as well as to the aging process. The techniques presented are broadly applicable in many diverse disease contexts and may be used in both diagnosis and research on new treatment strategies.