Results 31 to 40 of about 7,173 (197)
Effect of beta-Dystroglycan Processing on Utrophin / DP116 Anchorage in Normal and MDX Mouse Schwann Cell Membrane [PDF]
Neuroscience 141 (18/04/2006) 607-620, 2007 In the peripheral nervous system, utrophin and the short dystrophin isoform (Dp116) are co-localized at the outermost layer of the myelin sheath of nerve fibers; together with the dystroglycan complex. In peripheral nerve, matrix metalloproteinase (MMP) creates a 30 kDa fragment of beta-dystroglycan, leading to a disruption of the link between the ...
arxiv +1 more source
Nature Communications, 2022 Utrophin is a dystrophin-related protein stabilizing the sarcolemma in absence of dystrophin. Here the authors report that inactivation of the protein deacetylase SIRT6, involved in the deacetylation of the epigenetic mark H3K56ac in muscle cells ...
Angelina M. Georgieva +12 more
doaj +1 more source
Identification, Characterization, and Localization of a Novel Kidney Polycystin-1-Polycystin-2 Complex [PDF]
, 2002 The functions of the two proteins defective in autosomal dominant polycystic kidney disease, polycystin-1 and polycystin-2, have not been fully clarified, but it has been hypothesized that they may heterodimerize to form a "polycystin complex" involved ...
Ackermann +43 more
core +1 more source
Fundamental Research, 2022 Duchenne muscular dystrophy (DMD) is a serious genetic neuromuscular rare disease that is prevalent and caused by the mutation/deletion of the X-linked DMD gene that encodes dystrophin.
Ruo Wu +3 more
doaj
Utrophin influences mitochondrial pathology and oxidative stress in dystrophic muscle
Skeletal Muscle, 2017 Background Duchenne muscular dystrophy (DMD) is a lethal X-linked muscle wasting disorder caused by the absence of dystrophin, a large cytoskeletal muscle protein.
Tahnee L. Kennedy +6 more
doaj +1 more source
Scientific Reports, 2020 Upregulation of utrophin, a dystrophin related protein, is considered a promising therapeutic approach for Duchenne muscular dystrophy (DMD). Utrophin expression is repressed at the post-transcriptional level by a set of miRNAs, among which let-7c is ...
Kasturi Sengupta +2 more
doaj +1 more source
Cardioprotective Effect of Whole Body Periodic Acceleration in Dystrophic Phenotype mdx Rodent
Frontiers in Physiology, 2021 Duchenne muscular dystrophy (DMD) is characterized by progressive muscle wasting and the development of a dilated cardiomyopathy (DCM), which is the leading cause of death in DMD patients.
Arkady Uryash +5 more
doaj +1 more source
The Utrophin Gene Is Transcriptionally Up-regulated in Regenerating Muscle * [PDF]
, 2002 The utrophin gene codes for a large cytoskeletal protein closely related to dystrophin, the gene mutated in Duchenne's muscular dystrophy. Although utrophin could functionally substitute for dystrophin, in Duchenne's muscular dystrophy patients it did ...
Federico Galvagni +2 more
core +1 more source
PLoS ONE, 2016 Duchenne muscular dystrophy is a severe and currently incurable progressive neuromuscular condition, caused by mutations in the DMD gene that result in the inability to produce dystrophin. Lack of dystrophin leads to loss of muscle fibres and a reduction
Narinder Janghra +6 more
doaj +1 more source
Muscle structure influences utrophin expression in mdx mice. [PDF]
PLoS Genetics, 2014 Duchenne muscular dystrophy (DMD) is a severe muscle wasting disorder caused by mutations in the dystrophin gene. To examine the influence of muscle structure on the pathogenesis of DMD we generated mdx4cv:desmin double knockout (dko) mice.
Glen B Banks +4 more
doaj +1 more source