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Title Protein misfolding and disease : principles and protocols / edited by Peter Bross, Niels Gregersen
Published Totowa, N.J. : Humana Press, ©2003


Description 1 online resource (xv, 318 pages) : illustrations
Series Methods in molecular biology ; 232
Methods in molecular biology (Clifton, N.J.) ; v. 232
Contents 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 [and others] -- 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 [and others] -- 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 [and others] -- Investigation of folding and degradation of mutant proteins synthesized in semipermeabilized cells / C.M. Wilson and N.J. Bulleid
Summary It has now become clear that a large number of diseases with very different pathologies share a common framework of protein misfolding, accompanied by degradation and/or aggregation of the misfolded proteins. In Protein Misfolding and Disease: Principles and Protocols, notable experts in conformational disease review the latest thinking about the molecular processes underlying these diseases and describe cutting-edge 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 detailed research on new treatment strategies. Each tried and proven protocol includes insightful background notes, lists of required equipment and reagents, step-by-step instructions, and tips on troubleshooting and on how to avoid known pitfalls. Comprehensive and cutting-edge, Protein Misfolding and Disease: Principles and Protocols offers both novice and experienced researchers a solid theoretical grounding in conformational disease and a remarkable set of analytical methodologies for uncovering its characteristics and generating new therapeutic approaches
Bibliography Includes bibliographical references and index
Notes Print version record
Subject Proteins -- Pathophysiology -- Laboratory manuals
Protein folding -- Laboratory manuals
Proteins -- Conformation.
Protein Folding
Protein Conformation
MEDICAL -- Histology.
Proteins -- Conformation
Protein folding
Wetenschappelijke technieken.
Genre/Form Laboratory manuals
Laboratory manuals.
Manuels de laboratoire.
Form Electronic book
Author Bross, Peter.
Gregersen, Niels.
ISBN 1592593941