Abstract
Muscle degeneration and regeneration are two of the most evident pathological events characterizing muscular diseases and in particular muscular dystrophies. Muscular dystrophies are an heterogeneous group of hereditary diseases affecting both children and adults, and are characterized by muscle wasting and weakness. Until now at least 30 different genes have been associated with muscular dystrophies. They have been divided into several subgroups depending on the distribution of the muscle weakness. Thus, the histopathological markers of all these forms are dystrophic changes at the muscle biopsy characterized by fiber size variability, fibres necrosis, regeneration, inflammation and connective tissues deposition. As for now, no effective therapy is available for these diseases but new inside has now been expanded in regenerative therapy such as cell therapy and gene therapy. This review is focused on muscular dystrophies and new acknowledgments in regenerative therapy.
Keywords: Dystrophies, theraphy, exon skipping, LGMD, CMD
Current Pharmaceutical Design
Title: Muscular Dystrophies: Histology, Immunohistochemistry, Molecular Genetics and Management
Volume: 16 Issue: 8
Author(s): Costanza Lamperti and Maurizio Moggio
Affiliation:
Keywords: Dystrophies, theraphy, exon skipping, LGMD, CMD
Abstract: Muscle degeneration and regeneration are two of the most evident pathological events characterizing muscular diseases and in particular muscular dystrophies. Muscular dystrophies are an heterogeneous group of hereditary diseases affecting both children and adults, and are characterized by muscle wasting and weakness. Until now at least 30 different genes have been associated with muscular dystrophies. They have been divided into several subgroups depending on the distribution of the muscle weakness. Thus, the histopathological markers of all these forms are dystrophic changes at the muscle biopsy characterized by fiber size variability, fibres necrosis, regeneration, inflammation and connective tissues deposition. As for now, no effective therapy is available for these diseases but new inside has now been expanded in regenerative therapy such as cell therapy and gene therapy. This review is focused on muscular dystrophies and new acknowledgments in regenerative therapy.
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Cite this article as:
Lamperti Costanza and Moggio Maurizio, Muscular Dystrophies: Histology, Immunohistochemistry, Molecular Genetics and Management, Current Pharmaceutical Design 2010; 16 (8) . https://dx.doi.org/10.2174/138161210790883471
DOI https://dx.doi.org/10.2174/138161210790883471 |
Print ISSN 1381-6128 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4286 |
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