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Protein Misfolding and Disease, Principles and Protocols

12th April 2007

Protein Misfolding and Disease, Principles and Protocols

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ISBN: 1588290654 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.

Ed. Bross, Peter; Gregersen, Niels

2003; pp336; Methods in Molecular Biology, vol.232

 

Table of Contents

    1. Protein Misfolding, Aggregation, and Degradation in Disease (pp. 3-16)
      Gregersen, Niels; Bolund, Lars; Bross, Peter
    2. Basic Introduction to In Vivo Protein Folding and Its Defects (pp. 17-26)
      Bross, Peter; Gregersen, Niels
    3. Cystic Fibrosis: Premature Degradation of Mutant Proteins as a Molecular Disease Mechanism (pp. 27-38)
      Gelman, Marina S.; Kopito, Ron R.
    4. a1-Antitrypsin Deficiency: Liver Disease Associated With Retention of a Mutant Secretory Glycoprotein in the Endoplasmic Reticulum (pp. 39-56)
      Perlmutter, David H.
    5. Parkinson’s Disease: a -Synuclein and Parkin in Protein Aggregation and the Reversal of Unfolded Protein Stress (pp. 57-66)
      Jakobsen, Lene Diness; Jensen, Poul Henning
    6. Aberrant Protein Folding as the Molecular Basis of Cancer (pp. 67-76)
      Scott, Melissa D.; Frydman, Judith
    7. Yeast as a Model System to Investigate Protein Conformational Diseases (pp. 77-90)
      Coughlan, Christina M.; Brodsky, Jeffrey L.
    8. Expression of Recombinant Proteins: An Introduction (pp. 93-102)
      Nohr, Jane; Kristiansen, Karsten; Krogsdam, Anne-M.
    9. Expression in E. coli Systems (pp. 103-110)
      Krogsdam, Anne-M.; Kristiansen, Karsten; Nohr, Jane
    10. Protein Expression in Yeasts (pp. 111-126)
      Nohr, Jane; Kristiansen, Karsten; Krogsdam, Anne-M.
    11. Site-Directed Mutagenesis (pp. 127-132)
      Nohr, Jane; Kristiansen, Karsten
    12. Pulse-Chase Labeling Techniques for the Analysis of Protein Maturation and Degradation (pp. 133-146)
      Jansens, Annemieke; Braakman, Ineke
    13. Detection of Aggregates and Protein Inclusions by Staining of Tissues (pp. 149-164)
      Galvin, James E.
    14. Study of Mutant Proteins With Folding Defects in Cultured Patient Cells (pp. 165-174)
      Dodt, Gabriele; Walter, Claudia
    15. E. coli Expression System for Identifying Folding Mutations of Human Adenosine Deaminase (pp. 175-182)
      Santisteban, Ines; Arredondo-Vega, Francisco X.; Daniels, Shannon; Hershfield, Michael S.
    16. Characterization of Overexpressed Mutant Proteins in Mammalian Cells (pp. 183-202)
      Corydon, Thomas Juhl
    17. Investigation of Unfolded-Protein Response in Cells Expressing Familial Alzheimer’s Disease-Linked Presenilin Variants (pp. 203-216)
      Thinakaran, Gopal; Bowen, James W.; Ito, Daisuke; Leem, Jae-Yoon; Veselits, Margaret L.; Sato, Naoyuki
    18. Huntingtin Fragments Form Aggresome-Like Inclusion Bodies in Mammalian Cells (pp. 217-230)
      Boeddrich, Annett; Lurz, Rudi; Wanker, Erich E.
    19. Application of Chemical Chaperones to the Rescue of Folding Defects (pp. 231-244)
      Loo, Tip W.; Clarke, David M.
    20. Analysis of Defective Subunit Interactions Using the Two-Hybrid System (pp. 245-256)
      Perez, Belen; Desviat, Lourdes R.; Ugarte, Magdalena
    21. Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in the Cytosol (pp. 257-264)
      Desviat, Lourdes R.; Perez, Belen; Ugarte, Magdalena
    22. Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Microsomes (pp. 265-284)
      Cuthbert, Alan W.; Fuller, Will
    23. Investigation of Folding and Degradation of In Vitro Synthesized Mutant Proteins in Mitochondria (pp. 285-294)
      Bross, Peter; Winter, Vibeke; Pedersen, Christina Bak; Gregersen, Niels
    24. Investigation of Folding and Degradation of Mutant Proteins Synthesized in Semipermeabilized Cells (pp. 295-312)
      Wilson, Cornelia M.; Bulleid, Neil J.

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