Trinucleotide Repeat Protocols

  • Yoshinori Kohwi

Part of the Methods in Molecular Biology™ book series (MIMB, volume 277)

Table of contents

  1. Front Matter
    Pages i-xii
  2. Introduction

    1. Front Matter
      Pages 1-1
    2. Gillian P. Bates, David G. Hay
      Pages 3-15
  3. Analysis of Triplet Pepeat DNAs and RNAs

    1. Front Matter
      Pages 17-17
    2. Maria M. Krasilnikova, Sergei M. Mirkin
      Pages 19-28
    3. Michael J. Dixon, Saumitri Bhattacharyya, Robert S. Lahue
      Pages 29-45
    4. Mário Gomes-Pereira, Sanjay I. Bidichandani, Darren G. Monckton
      Pages 61-76
  4. Detection and Analysis of Polyglutamine-Containing Proteins and Their Aggregates

    1. Front Matter
      Pages 85-85
    2. Ali Khoshnan, Susan Ou, Jan Ko, Paul H. Patterson
      Pages 87-102
    3. Elizabeth Brooks, Montserrat Arrasate, Kenneth Cheung, Steven M. Finkbeiner
      Pages 103-128
  5. Establishment of Animal and Cultured Cell Models for Trinucleotide Repeat Diseases

    1. Front Matter
      Pages 139-139
    2. Violeta Stoyanova, Ben A. Oostra
      Pages 173-184
    3. Cédric Savouret, Claudine Junien, Geneviève Gourdon
      Pages 185-197
    4. Etienne Régulier, Diana Zala, Patrick Aebischer, Nicole Déglon
      Pages 199-213
    5. Mário Gomes-Pereira, Darren G. Monckton
      Pages 215-227
  6. In Vivo Analysis of Trinucleotide Repeat Diseases

    1. Front Matter
      Pages 229-229
    2. Caroline L. Benn, Laurie A. Farrell, Jang-Ho J. Cha
      Pages 231-260
    3. Melissa W. Braveman, Alice S. Chen-Plotkin, George J. Yohrling, Jang-Ho J. Cha
      Pages 261-276
    4. Tracie L. Moss, William O. Whetsell Jr.
      Pages 277-285
    5. Irina V. Kovtun, Craig Spiro, Cynthia T. McMurray
      Pages 309-319
    6. José Segovia, Francisca Pérez-Severiano
      Pages 321-334
  7. Back Matter
    Pages 335-342

About this book


The discovery that trinucleotide repeats significantly influence the age of onset and severity of a variety of hereditary neurological disorders has opened the door to a deeper understanding of the disease mechanisms involved, as well as to a more productive search for novel therapeutic interventions. In Trinucleotide Repeat Protocols, established leaders in trinucleotide repeat disease describe in step-by-step detail their best techniques for studying trinucleotide pathology at the molecular level. The protocols cover a variety of targets, ranging from DNA and RNA to proteins and whole animals, and focus not only on causal genes, but also on their consequent products, such as transcription factors, neurotransmitter receptors, proteasomes, and mitochondria/oxidation damage. Experimental systems employed include E. coli, yeast, C. elegans, mouse, and generally take a clinical point of view. The authors utilize a wide range of techniques, including gel electrophoresis, quantitative RT-PCR, immunological analysis, antibody usage and its applications, receptor assays using radioisotope handling, gene delivery by virus, brain cell and organotypic cultures, gender dependency, and neuron structure analysis. Each protocol follows the successful Methods in Molecular Biology™ series format, offering step-by-step laboratory instructions, an introduction outlining the principle behind the technique, lists of the necessary equipment and reagents, and tips on troubleshooting and avoiding known pitfalls.
Cutting-edge and highly practical, Trinucleotide Repeat Protocols offers neuroscientists powerful tools to elucidate both normal brain function and the mechanisms of hereditary neurological disease, as well as to develop the next generation of therapies for neuronal genetic diseases.

Editors and affiliations

  • Yoshinori Kohwi
    • 1
  1. 1.Life Science DivisionLawrence Berkeley LaboratoryBerkeleyCA

Bibliographic information

  • DOI
  • Copyright Information Humana Press 2004
  • Publisher Name Humana Press
  • eBook Packages Springer Protocols
  • Print ISBN 978-1-58829-243-8
  • Online ISBN 978-1-59259-804-5
  • Series Print ISSN 1064-3745
  • Series Online ISSN 1940-6029
  • Buy this book on publisher's site