Protein misfolding and disease : principles and protocols

Protein misfolding, aggregation, and degradation in disease / N. Gregersen, L. Bolund and P. Bross - - Basic introduction to in vivo protein folding and its defects / P. Bross and N. Gregersen - - Cystic fibrosis: Premature degradation of mutant proteins as a molecular disease mechanism / M.S. Gelman and R.R. Kopito - - Alpha1-antitrypsin deficiency: Liver disease associated with retention of a mutant secretory glycoprotein in the endoplasmic reticulum / D.H. Perlmutter - - Parkinson's disease: Alpha-synuclein and parkin in protein aggregation and the reversal of unfolded protein stress / L.D. Jakobsen and P.H. Jensen - - Aberrant protein folding as the molecular basis of cancer / M.D. Scott and J. Frydman - - Yeast as a model system to investigate protein conformational diseases / C.M. Coughlan and J.L. Brodsky - - Expression of recombinant proteins: An introduction / J. Nohr, K. Kristiansen and A.M. Krogsdam - - Expression in e. Coli systems / A.M. Krogsdam, K. Kristiansen and J. Nohr - - Protein expression in yeasts / J. Nohr, K. Kristiansen and A.M. Krogsdam - - Site-directed mutagenesis / J. Nohr and K. Kristiansen - - Pulse-chase labeling techniques for the analysis of protein maturation and degradation / A. Jansens and I. Braakman - - Detection of aggregates and protein inclusions by staining of tissues / J.E. Galvin - - Study of mutant proteins with folding defects in cultured patient cells / G. Dodt and C. Walter - - E. Coli expression system for identifying folding mutations of human adenosine deaminase / I. Santisteban ... [et al.] - - Characterization of overexpressed mutant proteins in mammalian cells / T.J. Corydon - - Investigation of unfolded-protein response in cells expressing familial alzheimer's disease-linked presenilin variants / G. Thinakaran ... [et al.] - - Huntingtin fragments form aggresome-like inclusion bodies in mammalian cells / A. Boeddrich, R. Lurz and E.E. Wanker - - Application of chemical chaperones to the rescue of folding defects / T.W. Loo and D.M. Clarke - - Analysis of defective subunit interactions using the two-hybrid system / B. Perez, L.R. Desviat and M. Ugarte - - Investigation of folding and degradation of in vitro synthesized mutant proteins in the cytosol / L.R. Desviat, B. Perez and M. Ugarte - - Investigation of folding and degradation of in vitro synthesized mutant proteins in microsomes / A.W. Cuthbert and W. Fuller - - Investigation of folding and degradation of in vitro synthesized mutant proteins in mitochondria / P. Bross ... [et al.] - - Investigation of folding and degradation of mutant proteins synthesized in semipermeabilized cells / C.M. Wilson and N.J. Bulleid