250 pages | 50 B/W Illus.
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.
1. Structure and Function of the Dystrophin-Glycoprotein Complex 2. The Structure and Function of Dystrophin 3. Utrophin in the Therapy of Duchenne Muscular Dystrophy 4. Syntrophin: A Molecular Adaptor Conferring a Signaling Role to the Dystrophin-Associated Protein Complex 5. Molecular and Functional Diversity of Dystrobrevin-Containing Complexes 6. Commonalities and Differences in Muscular Dystrophies: Mechanisms and Molecules Involved in Merosin-Deficient Congenital Muscular Dystrophy 7. Glycosylation and Muscular Dystrophy 8. Overview of the Limb-Girdle Muscular Dystrophies and Dysferlinopathy 9. Caveolin-3 and Limb-Girdle Muscular Dystrophy 10. The Sarcoglycans 11. Sarcomeric Proteins in LGMD 12. Lamins and Emerin in Muscular Dystrophy: The Nuclear Envelope Connection 13. Distinct Mechanisms Downstream of the Repeat Expansion Are Implicated in the Molecular Basis of Myotonic Dystrophy Type 1 14. Spinal Muscular Atrophy 15. The Pathophysiological Role of Impaired Calcium Handling in Muscular Dystrophy 16. Cell Adhesion and Signalling in the Muscular Dystrophies 17. Gene Therapies for Muscular Dystrophies 18. Cell Therapies for Muscular Dystrophy