Overview

Recent advances in molecular biological and associated cell biological technology have made skeletal muscle disease a particularly important and exciting area of research. ""Molecular and Cell Biology of Muscular Dystrophy"" gives a series of accounts on various aspects of the remarkable breakthrough which has been achieved in our understanding of the Duchenne/Becker muscular dystrophies and of the consequences and ramifications of this breakthrough. A number of articles address the matter of how molecular biological technology can advance the cell biology of skeletal muscle and the possible applications of this knowledge of therapy. Current research literature describing the advances being made in molecular and cell biological research is quite incomprehensible to the majority of clinical neurologists, paediatricians and many basic scientists with marginal interests in muscle disease, being based on disciplines remote from their training and being heavily laden with acronyms and jargon. This book attempts to remedy this problem by presenting the prototype example of the ""reverse genetic"" approach as applied to Duchenne/Becker muscular dystrophy in a generally intelligible manner and by exploring its far-reaching practical consequences in both clinical diagnosis and in research. ""Molecular and Cell Biology of Muscular Dystrophy"" provides access to the topic for academics from a number of disciplines in which muscle function and malfunction is a major interest, eg neurologists, paediatricians, physiologists, biochemists and biologists. This book should be of interest to graduate students and researchers in clinical biochemistry, molecular medicine, neuroscience and clinicians in neurology.

Full Product Details

Author:   T. Partridge
Publisher:   Chapman and Hall
Imprint:   Chapman and Hall
Edition:   1993 ed.
Dimensions:   Width: 15.50cm , Height: 2.00cm , Length: 23.50cm
Weight:   1.510kg
ISBN:  

9780412434402

ISBN 10:   0412434407
Pages:   344
Publication Date:   31 July 1993
Audience:   College/higher education ,  Professional and scholarly ,  Postgraduate, Research & Scholarly ,  Professional & Vocational
Format:   Hardback
Publisher's Status:   Active
Availability:   In Print  
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Table of Contents

1 Molecular human genetics and the Duchenne/Becker muscular dystrophy gene.- 1.1 Introduction.- 1.2 DMD and BMD phenotype.- 1.3 Localization to Xp21.- 1.4 Isolation of Xp21 sequences.- 1.5 Chromosome walking and jumping in Xp21.- 1.6 Identification of expressed sequences.- 1.7 Gene sequence and organization.- 1.8 Genotype and phenotype.- 1.9 Dystrophin.- 1.10 Conclusions.- References.- 2 Genotype/phenotype correlations in Duchenne/Becker dystrophy.- 2.1 Introduction.- 2.2 Duchenne muscular dystrophy.- 2.3 Dystrophinopathy in girls and women.- 2.4 Becker muscular dystrophy.- 2.5 Conclusion.- References.- 3 Molecular genetics and genetic counselling for Duchenne/Becker muscular dystrophy.- 3.1 Introduction.- 3.2 Duchenne and Becker muscular dystrophy.- 3.3 Genetic counselling for Duchenne and Becker muscular dystrophy: prior to molecular genetics.- 3.4 Genetic counselling of Duchenne and Becker muscular dystrophy post molecular genetics.- 3.5 Genetic counselling of Duchenne and Becker muscular dystrophy: Future.- 3.6 Conclusion.- Acknowledgements.- References.- 4 Inheritance and pathogenicity of myotonic dystrophy.- 4.1 Introduction.- 4.2 The clinical picture.- 4.3 Biochemical, electrophysiological and histological findings.- 4.4 Genetic mapping of the myotonic locus.- 4.5 Physical mapping.- 4.6 Isolation of the DM region as a series of overlapping genomic clones.- 4.7 Identifying candidate genes.- 4.8 Future prospects.- Acknowledgements.- References.- 5 Genetic mapping of facioscapulohumeral muscular dystrophy.- 5.1 Introduction.- 5.2 Positional cloning.- 5.3 Facioscapulohumeral muscular dystrophy: clinical features.- 5.4 FSHD: Linkage analysis and positional cloning.- Acknowledgements.- References.- 6 Dystrophin-associated glycoproteins: their possible roles in the pathogenesis of Duchenne muscular dystrophy.- 6.1 Introduction.- 6.2 Membrane properties of dystrophin.- 6.3 Dystrophin—glycoprotein complex.- 6.4 Structure and function of dystroglycan (43/156 dystrophin-associated glycoprotein).- 6.5 Molecular pathogenesis of Duchenne muscular dystrophy.- 6.6 Summary.- Acknowledgements.- References.- 7 PCR analysis of muscular dystrophy in mdx mice.- 7.1 Introduction.- 7.2 Mouse dystrophin cDNA clones.- 7.3 mdx mice.- 7.4 PCR analysis of mouse dystrophin expression.- 7.5 Summary.- Acknowledgements.- References.- 8 Cell biology of the satellite cell.- 8.1 Introduction.- 8.2 Satellite cells in normal muscle.- 8.3 Behaviour of satellite cells in damaged muscle.- 8.4 Satellite cells, myoblasts and implant therapy.- References.- 9 Molecular and cell biology of skeletal muscle regeneration.- 9.1 Introduction.- 9.2 Developmental origin of satellite cells and their identification in vivo.- 9.3 Replication during postnatal growth.- 9.4 Numbers and proliferative capacity of mpc from animals of different ages.- 9.5 The influence of growth factors and extracellular matrix components on the proliferation and differentiation of mpc.- Acknowledgements.- References.- 10 Molecular mechanisms of muscle damage.- 10.1 Introduction.- 10.2 The mechanisms of damage to normal skeletal muscle subjected to various damaging stresses.- 10.3 Mechanisms of damage in dystrophin-deficient muscle.- 10.4 Conclusions.- Acknowledgements.- References.- 11 Human dystrophin gene transfer: Genetic correction of dystrophin deficiency.- 11.1 Somatic gene therapy: An introduction.- 11.2 Therapeutic targets in Duchenne muscular dystrophy.- 11.3 Recombinant dystrophin genes: Their nature and availability.- 11.4 Physical gene transfer techniques.- 11.5 Viral-mediated gene transferstrategies.- 11.6 Studies in transgenic animals: Germ line gene transfer.- 11.7 Dystrophin: Structure and function.- 11.8 Gene therapy for Duchenne muscular dystrophy: Future prospects.- References.- 12 Myoblast transplantation in inherited myopathies.- 12.1 Introduction.- 12.2 Myoblast transplantation in animal models of inherited myopathies.- 12.3 Problems associated with myoblast transfer.- Acknowledgements.- References.

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