Results 31 to 40 of about 7,812 (251)
Sarcospan Regulates Cardiac Isoproterenol Response and Prevents Duchenne Muscular Dystrophy-Associated Cardiomyopathy. [PDF]
, 2015 BackgroundDuchenne muscular dystrophy is a fatal cardiac and skeletal muscle disease resulting from mutations in the dystrophin gene. We have previously demonstrated that a dystrophin-associated protein, sarcospan (SSPN), ameliorated Duchenne muscular ...
Crosbie-Watson, Rachelle H +6 more
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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
A study of short utrophin isoforms in mice deficient for full-length utrophin
Mammalian Genome, 2003 Utrophin can functionally replace dystrophin in dystrophin-deficient muscle and may have a role in a therapeutic strategy for Duchenne muscular dystrophy. This has resulted in many investigations of the full-length muscle form of utrophin; however, the short utrophins and non-muscle forms have been relatively neglected, partly because they are ...
Kay E. Davies +3 more
openaire +4 more sources
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
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
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
Science-Business eXchange, 2011 Tivorsan Pharmaceuticals is working with a Brown University team to optimize recombinant biglycan for delivery to patients with Duchenne muscular dystrophy. The work builds on the researchers' finding that the extracellular matrix glycoprotein decreases muscle pathology and improves muscle function in dystrophic mice.
openaire +2 more sources
Cloning and expression of full length mouse utrophin: the differential association of utrophin and dystrophin with AChR clusters [PDF]
FEBS Letters, 1996 We have cloned and sequenced mouse utrophin cDNA, and successfully expressed full length utrophin (400 kDa) in both muscle and non‐muscle cells. The expression of recombinant utrophin is compared with that of its homologue, dystrophin (427 kDa). We demonstrate that recombinant utrophin is targeted into agrin‐induced acetylcholine receptor (AChR ...
W.-X. Athena Guo +2 more
openaire +3 more sources
Revertant fibres and dystrophin traces in Duchenne muscular dystrophy: Implication for clinical trials [PDF]
, 2010 Duchenne muscular dystrophy (DMD) is characterised by the absence of dystrophin in muscle biopsies, although residual dystrophin can be present, either as dystrophin-positive (revertant) fibres or traces.
Arechavala-Gomeza, V +12 more
core +1 more source