Micro-utrophin Improves Cardiac and Skeletal Muscle Function of Severely Affected D2/mdx Mice
Molecular Therapy: Methods & Clinical Development, 2018 Duchenne muscular dystrophy (DMD) is an X-linked muscle-wasting disease caused by mutations in the dystrophin gene. DMD boys are wheelchair-bound around 12 years and generally survive into their twenties.
Tahnee L. Kennedy +10 more
doaj
Employment of Microencapsulated Sertoli Cells as a New Tool to Treat Duchenne Muscular Dystrophy
Journal of Functional Morphology and Kinesiology, 2017 Duchenne muscular dystrophy (DMD) is a lethal X-linked pathology due to lack of dystrophin and characterized by progressive muscle degeneration, impaired locomotion and premature death.
Sara Chiappalupi +6 more
doaj +1 more source
WH2 and proline-rich domains of WASP-family proteins collaborate to accelerate actin filament elongation. [PDF]
, 2017 WASP-family proteins are known to promote assembly of branched actin networks by stimulating the filament-nucleating activity of the Arp2/3 complex.
Akin, Orkun +6 more
core +1 more source
Disruption of the utrophin‒actin interaction by monoclonal antibodies and prediction of an actin-binding surface of utrophin [PDF]
, 1999 Glenn E. Morris +5 more
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Regulation of the cardiac sodium channel Nav1.5 by utrophin in dystrophin-deficient mice [PDF]
, 2017 Aims Duchenne muscular dystrophy (DMD) is a severe striated muscle disease due to the absence of dystrophin. Dystrophin deficiency results in dysfunctional sodium channels and conduction abnormalities in hearts of mdx mice. Disease progression in the mdx
Abriel, Hugues +3 more
core
Description of a utrophin associated protein complex in lipid raft domains of human artery smooth muscle cells [PDF]
, 2014 The dystrophin-associated protein complex (DAPC) is a multimeric complex that links the extracellular matrix to the actin cytoskeleton, and in some cases dystrophin can be substituted by its autosomal homologue utrophin to form the utrophin-associated ...
Canto, Patricia +4 more
core +1 more source
G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain. [PDF]
, 1995 Derek J. Blake +7 more
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Local Transcriptional Control of Utrophin Expression at the Neuromuscular Synapse [PDF]
, 1997 Anthony O. Gramolini +6 more
openalex +1 more source
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
core +2 more sources
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
doaj +1 more source