Get started
Work

Molecular Pathological Study on Interstrain Differences in the Skeletal Muscle Lesions between Dysferlin-deficient SJL and A/J Mice

Year: 2009
Type: dissertation
Abstract: Muscular dystrophy is a generic term used to refer to as hereditary muscle disorder characterized clinically in progressive muscle weakness and muscle atrophy, and histopathologically in degeneration/necrosis and regeneration of skeletal muscle fibers.The progression of disease confines patients to a wheelchair or ventilator, and detracts from quality of life (QOL).Although various approaches to therapy of muscular dystrophy, for example, gene therapy including exon skipping or cell based therapy (Arnett et al., 2009;Farini et al., 2009;Wang et al., 2009), are just sought, the standard therapies remain to be established.This group of inherited muscle disorders includes Duchenne muscular dystrophy, Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy, limb girdle muscular dystrophies, distal muscular dystrophies (Miyoshi myopathy, tibial muscular dystrophy, myofibrillary/desmin-related myopathy, Welander myopathy et al.), Fukuyama/non-Fukuyama congenital muscular dystrophies, Nonakel myopathy (also known as distal myopathy with rimmed vacuoles), facioscapulohumeral muscular dystrophy, myotonic dystrophy et al. (Cohn and Campbell, 2000).Out of these, limb girdle muscular dystrophy (LGMD) is a muscle disorder which weakness and muscle atrophy progress slowly and symmetrically in the proximal muscles of limb girdles.Because LGMD included many unclassified muscular dystrophies and were etiologically heterogeneous, a locus-based classification was proposed by a consortium meeting under the auspices of the European Neuromuscular Centre, with the dominant LGMD loci designated LGMD1A, B, C, etc. and the recessive froms as LGMD2A, B, C, etc. in the order of their identification (Bushby, 1999).
DOI: 10.24729/00000558
Language: English
Open Access status: green
OpenAlex ID: W2188449444
Cites: 63
Cited by:
Related to: 10