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Molecular Mechanisms of Muscular Dystrophies


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Steve J. Winder
University of Sheffield
Sheffield, U.K.

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ISBN: 978-1-58706-264-3
Pub date: 2006-01-26
250 pages
50 figures
13 tables


About this book

There is no doubt that the study of the muscular dystrophies in recent years has been exciting and rewarding. It has attracted the attention of many investigators of international repute, and this is reflected in the various contributions to this volume. Molecular Mechanisms of Muscular Dystrophies represents a personal collection of chapters covering a spectrum of muscular dystrophies and one myopathy that represent most of the major muscular dystrophies, and, in particular, those where a molecular understanding of the underlying mechanisms is most advanced, rather than a comprehensive treatise on every muscular dystrophy so far characterized.

Table of contents

1. Structure and Function of the Dystrophin-Glycoprotein Complex
James M. Ervasti

2. The Structure and Function of Dystrophin
Simone Abmayr and Jeff Chamberlain

3. Utrophin in the Therapy of Duchenne Muscular Dystrophy
Qing Bai, Edward A. Burton and Kay E. Davies

4. Syntrophin: A Molecular Adaptor Conferring a Signaling Role to the Dystrophin-Associated
Protein Complex
Justin M. Percival, Marvin E. Adams and Stanley C. Froehner

5. Molecular and Functional Diversity of Dystrobrevin-Containing Complexes
Derek J. Blake and Roy V. Sillitoe

6. Commonalities and Differences in Muscular Dystrophies: Mechanisms and Molecules Involved
in Merosin-Deficient Congenital Muscular Dystrophy
Markus A. Ruegg

7. Glycosylation and Muscular Dystrophy
Susan C. Brown and Francesco Muntoni

8. Overview of the Limb-Girdle Muscular Dystrophies and Dysferlinopathy
Kate M.D. Bushby and S.H. Laval

9. Caveolin-3 and Limb-Girdle Muscular Dystrophy
Ferruccio Galbiati and Michael P. Lisanti

10. The Sarcoglycans
Elizabeth M. McNally

11. Sarcomeric Proteins in LGMD
Olli Carpén

12. Lamins and Emerin in Muscular Dystrophy: The Nuclear Envelope Connection
Josef Gotzmann and Roland Foisner

13. Distinct Mechanisms Downstream of the Repeat Expansion Are Implicated in the Molecular Basis Myotonic Dystrophy Type 1
Keith Johnson and Rami Jarjour

14. Spinal Muscular Atrophy
Robert Olaso, Jérémie Vitte, Nouzha Salah and Judith Melki

15. The Pathophysiological Role of Impaired Calcium Handling in Muscular Dystrophy
Kay Ohlendieck

16. Cell Adhesion and Signalling in the Muscular Dystrophies
Steve J. Winder

17. Gene Therapies for Muscular Dystrophies
Dominic J. Wells

18. Cell Therapies for Muscular Dystrophy
Terence A. Partridge