The spectrin family of proteins: a unique coiled-coil fold for various molecular surface properties
, 2014 International audienceThe spectrin superfamily is composed of proteins involved in cytolinker functions. Their main structural feature is a large central subdomain with numerous repeats folded in triple helical coiled-coils.
Delalande, Olivier +3 more
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Duchenne muscular dystrophy: overview and future challenges Dystrofia = mięśniowa Duchenne’a: przegląd literatury i wyzwania w przyszłości [PDF]
, 2017 Duchenne muscular dystrophy is a muscle disease caused by mutation in the gene that encodes the cytoskeletal protein dystrophin. It is inherited in an X-linked recessive fashion.
Basuki, Mudjiani +3 more
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256. An Ideal Therapeutic Agent for Duchenne Muscular Dystrophy Involving a Gutted Adenovirus Expressing Full-Length Utrophin [PDF]
, 2005 Jatinderpal R. Deol +5 more
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The Journal of Cell Biology, 2002 ![Graphic][1] Utrophin (green) can bind to actin (light blue) in two different modes.Utrophin, a member of the spectrin superfamily of actin-binding proteins ubiquitously expressed in human cells, helps link the actin cytoskeleton to the extracellular matrix. Galkin et al.
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Neurobiology of Disease, 2014 Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease caused by lack of dystrophin, a sub-sarcolemmal protein, which leads to dramatic muscle deterioration.
Sara Vianello +8 more
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A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice. [PDF]
, 2001 Dystrophin-deficient muscles experience large reductions in expression of nitric oxide synthase (NOS), which suggests that NO deficiency may influence the dystrophic pathology.
Spencer, MJ, Tidball, JG, Wehling, M
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Expression and function of utrophin associated protein complex in stretched endothelial cells: dissociation and activation of eNOS [PDF]
, 2006 Israel Ramírez‐Sánchez +6 more
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The dystrotelin, dystrophin and dystrobrevin superfamily: new paralogues and old isoforms
BMC Genomics, 2007 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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Prevention of pathology in mdx mice by expression of utrophin: analysis using an inducible transgenic expression system [PDF]
, 2002 Sarah Squire +7 more
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Arquivos de Neuro-PsiquiatriaSignificant advances in the understanding and management of Duchenne muscular dystrophy (DMD) took place since international guidelines were published in 2010.
Alexandra P. Q. C. Araujo +15 more
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