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Gentamicin fails to increase dystrophin expression in dystrophin‐deficient muscle
Muscle & Nerve, 2003Abstract A recent report that aminoglycoside antibiotics restored the expression of functional dystrophin to skeletal muscles of mdx mice, a model of Duchenne muscular dystrophy (DMD), raised hopes that DMD may be treatable by a conventional drug. Subsequently, several human
Patrick, Dunant +3 more
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[Dystrophin, dystrophin-associated protein and dystrophinopathy].
Nihon shinkei seishin yakurigaku zasshi = Japanese journal of psychopharmacology, 1995Dystrophin is a protein product of the gene responsible for Duchenne muscular dystrophy (DMD), and is a long slender protein localized at the protoplasmic surface of sarcolemma. Dystrophin binds with actin filaments at its amino-terminal region, and with dystrophin-associated proteins (DAPs) at its carboxyl-terminal region.
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Dystrophin: A clinical perspective
Pediatric Neurology, 1990Dystrophin, the protein product of the gene related to Duchenne and Becker muscular dystrophies, is a large cytoskeletal protein associated with the muscle fiber membrane. Recently identified dystrophin-related myopathies affecting animals can serve as experimental models for human disease.
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Human dystrophin gene transfer: genetic correction of dystrophin deficiency
1993Somatic gene therapy, as generally conceived, involves reconstituting a biological function by adding a normal gene to somatic, i.e. non-germline, cells which are genetically deficient in that gene product (Friedmann, 1989). Single gene or Mendelian disorders which are recessive (autosomal or X-linked) are particularly attractive candidates for such a ...
G, Dickson, M, Dunckley
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Hippocampal LTP in dystrophin and dystrophin isoforms deficient mice.
1997info:eu-repo/semantics ...
Godfraind, J M +2 more
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Manual Immunofluorescence Staining for Dystrophin and Key Dystrophin-Associated Proteins
The detection of dystrophin and the proteins of the dystrophin-associated protein complex (DAPC) by immunostaining allows for the detection of the presence and localization of these proteins in slide-mounted sections of skeletal muscle or heart tissue. This technique has been used historically for the diagnosis and characterization of muscle disorders ...Hui, Meng +5 more
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Dystrophin and Dystrophin-Associated Proteins
2014I. Kleyman, T.H. Brannagan
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