منابع مشابه
Mechanisms of muscle weakness in muscular dystrophy
Disruption of dystrophin or the sarcoglycans within the dystrophin protein complex leads to muscular dystro-phy. The dystrophin complex is enriched in myofibers and concentrated into costameres, rib-like structures in the plasma membrane. In the absence of dystrophin, the plasma membrane becomes fragile with reduced stiffness and increased leakiness, and exposing dystro-phin-deficient muscle to...
متن کاملMuscular Dystrophy: Disease Mechanisms and Therapies
Progressive weakness and degeneration of skeletal muscles caused by genetic alterations fall into the category of muscular dystrophy. Muscular dystrophy occurs worldwide and affects all races. The overall incidence of muscular dystrophy varies among forms, as some forms are more common than others. Muscle loss and weakness are not necessarily caused by genetic alteration. Skeletal muscle inacti...
متن کاملCellular and molecular mechanisms underlying muscular dystrophy
The muscular dystrophies are a group of heterogeneous genetic diseases characterized by progressive degeneration and weakness of skeletal muscle. Since the discovery of the first muscular dystrophy gene encoding dystrophin, a large number of genes have been identified that are involved in various muscle-wasting and neuromuscular disorders. Human genetic studies complemented by animal model syst...
متن کاملP164: Adeno-Associated Viral Vectors in Duchenne Muscular Dystrophy
Duchenne muscular dystrophy (BMD) is an inherited X-link disease. The incidence of this muscle-wasting disease is 1:5000 male live births. Mutation in the gene coding for dystrophin is the main cause of BMD. Most cases of this disease succumb to respiratory and cardiac failure in 3rd to 4th decades. The slow progression of BMD and recent achievement of gene therapies make it as an appropriate c...
متن کاملSatellite Cells: Regenerative Mechanisms and Applicability in Muscular Dystrophy
The satellite cells are long regarded as heterogeneous cell population, which is intimately linked to the processes of muscular recovery. The heterogeneous cell population may be classified by specific markers. In spite of the significant amount of variation amongst the satellite cell populations, it seems that their activity is tightly bound to the paired box 7 transcription factor expression,...
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ژورنال
عنوان ژورنال: The International Journal of Biochemistry & Cell Biology
سال: 2013
ISSN: 1357-2725
DOI: 10.1016/j.biocel.2013.05.001