Halofuginone improves muscle-cell survival in muscular dystrophies
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چکیده
منابع مشابه
Muscle MRI in muscular dystrophies.
Muscle MRI has become a very useful tool in the diagnosis and follow-up of patients with muscle dystrophies. Muscle MRI provides us about many aspects of the structure and function of skeletal muscles, such as the presence of oedema or fatty infiltration. In the last years many reports have described the particular muscles that are involved in these muscle disease. This knowledge can facilitate...
متن کاملMuscle diseases: the muscular dystrophies.
Dystrophic muscle disease can occur at any age. Early- or childhood-onset muscular dystrophies may be associated with profound loss of muscle function, affecting ambulation, posture, and cardiac and respiratory function. Late-onset muscular dystrophies or myopathies may be mild and associated with slight weakness and an inability to increase muscle mass. The phenotype of muscular dystrophy is a...
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Skeletal muscles are the agent of motion and one of the most important tissues responsible for the control of metabolism. The maintenance of muscle homeostasis is finely regulated by the balance between catabolic and anabolic process. Macroautophagy (or autophagy) is a catabolic process that provides the degradation of protein aggregation and damaged organelles through the fusion between autoph...
متن کاملMuscular dystrophies, the cytoskeleton and cell adhesion.
Muscular dystrophies are associated with mutations in genes encoding several classes of proteins. These range from extracellular matrix and integral membrane proteins to cytoskeletal proteins, but also include a heterogeneous group of proteins including proteases, nuclear proteins, and signalling molecules. Muscular dystrophy phenotypes have also become evident in studies on various knockout mi...
متن کاملMuscle exercise in limb girdle muscular dystrophies: pitfall and advantages
Different genetic mutations underlying distinct pathogenic mechanisms have been identified as cause of muscle fibers degeneration and strength loss in limb girdle muscular dystrophies (LGMD). As a consequence, exercise tolerance is affected in patients with LGMD, either as a direct consequence of the loss of muscle fibers or secondary to the sedentary lifestyle due to the motor impairment. It h...
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ژورنال
عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Molecular Cell Research
سال: 2014
ISSN: 0167-4889
DOI: 10.1016/j.bbamcr.2014.03.025