Utrophin up-regulation helps maintain normal cardiac geometry in a gene therapy model for Duhenne muscular dystrophy heart disease [abstract] [PDF]
Duchenne muscular dystrophy (DMD) is the most common childhood muscle wasting disease. DMD sufferers rarely survive past their mid-twenties succumbing to respiratory or heart failure.
Bostick, Brian P., 1979-
core
Utrophin: A Structural and Functional Comparison to Dystrophin [PDF]
Derek J. Blake+2 more
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l-arginine improves dystrophic phenotype in mdx mice
A possible treatment for Duchenne muscular dystrophies would be to compensate for dystrophin loss by increasing the expression of utrophin, another cytoskeletal protein of the muscle membrane. We previously found that l-arginine, the substrate for nitric
Vincent Voisin+7 more
doaj
The dystrotelin, dystrophin and dystrobrevin superfamily: new paralogues and old isoforms
Background Dystrophins and dystrobrevins are distantly related proteins with important but poorly understood roles in the function of metazoan muscular and neuronal tissues.
Hughes Simon M+10 more
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Adhesion-dependent tyrosine phosphorylation of β-dystroglycan regulates its interaction with utrophin [PDF]
Marian James+6 more
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Regulation and functional significance of utrophin expression at the mammalian neuromuscular synapse [PDF]
Anthony O. Gramolini+2 more
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Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure [PDF]
Jill A. Rafael
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Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism. [PDF]
Biological roles of obscurin and its close homolog Obsl1 (obscurin-like 1) have been enigmatic. While obscurin is highly expressed in striated muscles, Obsl1 is found ubiquitously.
Blondelle, Jordan+13 more
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Sp1 and Sp3 physically interact and co-operate with GABP for the activation of the utrophin promoter [PDF]
Federico Galvagni+2 more
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iNOS Expression In Dystrophinopathies Can Be Reduced By Somatic Gene Transfer of Dystrophin or Utrophin [PDF]
Jean‐Pierre Louboutin+4 more
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