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
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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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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
Molecular heterogeneity of the dystrophin-associated protein complex in the mouse kidney nephron: differential alterations in the absence of utrophin and dystrophin [PDF]
, 2018 The dystrophin-associated protein complex (DPC) consisting of syntrophin, dystrobrevin, and dystroglycan isoforms is associated either with dystrophin or its homolog utrophin.
Fritschy, Jean-Marc +2 more
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Clustering of nicotinic acetylcholine receptors: from the neuromuscular junction to interneuronal synapses [PDF]
, 2018 Fast and accurate synaptic transmission requires high-density accumulation of neurotransmitter receptors in the postsynaptic membrane. During development of the neuromuscular junction, clustering of acetylcholine receptors (AChR) is one of the first ...
Fuhrer, C., Huh, K H.
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Utrophin up-regulation helps maintain normal cardiac geometry in a gene therapy model for Duhenne muscular dystrophy heart disease [abstract] [PDF]
, 2010 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-
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Utrophin, MHC and M1/M2 macrophages in GRMD dogs
Ciência Animal Brasileira, 2020 Muscular dystrophies are hereditary diseases that lead to progressive degeneration of the skeletal musculature. Golden Retriever dogs are used as animal models because they show a hereditary muscle disease similar to muscular dystrophy in humans.
Gabriela Noronha de Toledo +1 more
doaj
Utrophin upregulation in Duchenne muscular dystrophy.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology, 2006 Duchenne Muscular Dystrophy (DMD) is a devastating, progressive muscle wasting disease for which there is currently no effective treatment. DMD is caused by mutations in the dystrophin gene many of which result in the absence of the large cytoskeletal protein dystrophin at the sarcolemma.
Hirst, R, McCullagh, K, Davies, K
openaire +2 more sources
In vitro stability of therapeutically relevant, internally truncated dystrophins [PDF]
, 2015 Dana M Talsness +5 more
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