Molecular Mechanisms of Muscular Dystrophies

ISBN-13: 9781587062643 / Angielski / Twarda / 2006 / 250 str.

Steve J. Winder

Opis

This book presents a 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.

Spis treści

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

O autorze

Winder\, Steve J.