Protein Misfolding and Disease

General Concepts and Models.- Protein Misfolding, Aggregation, and Degradation in Disease.- Basic Introduction to In Vivo Protein Folding and Its Defects.- Cystic Fibrosis.- ?1-Antitrypsin Deficiency.- Parkinson’s Disease.- Aberrant Protein Folding as the Molecular Basis of Cancer.- Yeast as a Model System to Investigate Protein Conformational Diseases.- General Methods.- Expression of Recombinant Proteins.- Expression in E. coli Systems.- Protein Expression in Yeasts.- Site-Directed Mutagenesis.- Pulse-Chase Labeling Techniques for the Analysis of Protein Maturation and Degradation.- Techniques in Conformational Disease Research.- Detection of Aggregates and Protein Inclusions by Staining of Tissues.- Study of Mutant Proteins With Folding Defects in Cultured Patient Cells.- E. coli Expression System for Identifying Folding Mutations of Human Adenosine Deaminase.- Characterization of Overexpressed Mutant Proteins in Mammalian Cells.- Investigation of Unfolded-Protein Response in Cells Expressing Familial Alzheimer's Disease-Linked Presenilin Variants.- Huntingtin Fragments Form Aggresome-Like Inclusion Bodies in Mammalian Cells.- Application of Chemical Chaperones to the Rescue of Folding Defects.- Analysis of Defective Subunit Interactions Using the Two-Hybrid System.- Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in the Cytosol.- Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Microsomes.- Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Mitochondria.- Investigation of Folding and Degradation of Mutant Proteins Synthesized in Semipermeabilized Cells.