Special Issue—Towards Understanding the Mechanisms and Curing of Muscular Dystrophy Diseases
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منابع مشابه
Special Issue--Towards Understanding the Mechanisms and Curing of Muscular Dystrophy Diseases.
Muscular dystrophies are a heterogeneous group of inherited diseases with different molecular basss, but sharing similar clinical features and dystrophic changes.
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 کاملMechanisms 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 کاملUnderstanding the Process of Fibrosis in Duchenne Muscular Dystrophy
Fibrosis is the aberrant deposition of extracellular matrix (ECM) components during tissue healing leading to loss of its architecture and function. Fibrotic diseases are often associated with chronic pathologies and occur in a large variety of vital organs and tissues, including skeletal muscle. In human muscle, fibrosis is most readily associated with the severe muscle wasting disorder Duchen...
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ژورنال
عنوان ژورنال: Molecules
سال: 2015
ISSN: 1420-3049
DOI: 10.3390/molecules200712944