Dystrophin : gene, protein, and cell biology

著者

    • Brown, Susan C.
    • Lucy, Jack A.

書誌事項

Dystrophin : gene, protein, and cell biology

edited by Susan C. Brown and Jack A. Lucy

Cambridge University Press, 1997

大学図書館所蔵 件 / 5

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注記

Includes bibliographical references and index

内容説明・目次

内容説明

This book is concerned with advances in research on dystrophin, and how its absence gives rise to muscular dystrophy. It is the first book to address relationships between the molecular structure and function of dystrophin since the structure of the gene for this protein was elucidated in 1988. The volume covers recent advances in knowledge on the structure of both the dystrophin gene and its associated promoters, and on the protein itself. Functional interactions of dystrophin and its related proteins in the environment of the plasma membrane are a central feature of the book. Other aspects considered are the expression of the dystrophin complex in muscle, in the brain, and at the neuromuscular junction. The book concludes with discussions of muscle regeneration, gene therapy of Duchenne muscular dystrophy, and cellular approaches to the therapy of the disease.

目次

  • Foreword Martin Bobrow
  • 1. Structure and mutation of the dystrophin gene Andrea Amalfitano, Jill A. Rafael and Jeffrey S. Chamberlain
  • 2. Protein structure S. J. Winder, A. E. Knight and J. Kendrick-Jones
  • 3. Dystrophin, utrophin and their associated proteins J. M. Tinsley, D. J. Blake, and K. E. Davies
  • 4. Localization of dystrophin in skeletal, cardiac and smooth muscle Simon C. Watkins, Darl R Swartz and Timothy J. Byers
  • 5. Expression of the dystrophin complex in the brain Dariusz C. Gorecki and Eric A. Barnard
  • 6. The dystrophin family, agrin, and construction of the neuromuscular junction Robert Sealock and Stanley Froehner
  • 7. Functions of dystrophin Susan C. Brown and Jack A. Lucy
  • 8. Pathophysiology of dystrophin deficiency: a clinical and biological enigma J. Rafael M. Gorospe, Bronwen K. Nishikawa and Eric P. Hoffman
  • 9. Muscle regeneration M. J. Cullen
  • 10. Gene therapy of Duchenne muscular dystrophy Gaynor Turner, Matthew G. Dunckley and George Dickson
  • 11. Models of dystrinopathy, pathological mechanisms, and therapies T. A. Partridge
  • Index.

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