IRES-mediated translation of utrophin A is enhanced by glucocorticoid treatment in skeletal muscle cells. [PDF]
PLoS ONE, 2008 Glucocorticoids are currently the only drug treatment recognized to benefit Duchenne muscular dystrophy (DMD) patients. The nature of the mechanisms underlying the beneficial effects remains incompletely understood but may involve an increase in the ...
Pedro Miura +3 more
doaj +5 more sources
Drug discovery for Duchenne muscular dystrophy via utrophin promoter activation screening. [PDF]
PLoS ONE, 2011 Duchenne muscular dystrophy (DMD) is a devastating muscle wasting disease caused by mutations in dystrophin, a muscle cytoskeletal protein. Utrophin is a homologue of dystrophin that can functionally compensate for its absence when expressed at increased
Catherine Moorwood +5 more
doaj +6 more sources
Translational regulation of utrophin by miRNAs. [PDF]
PLoS ONE, 2011 BackgroundUtrophin is the autosomal homolog of dystrophin, the product of the Duchenne Muscular Dystrophy (DMD) locus. Its regulation is of therapeutic interest as its overexpression can compensate for dystrophin's absence in animal models of DMD.
Utpal Basu +5 more
doaj +6 more sources
Rescue of dystrophic skeletal muscle by PGC-1α involves a fast to slow fiber type shift in the mdx mouse. [PDF]
PLoS ONE, 2012 Increased utrophin expression is known to reduce pathology in dystrophin-deficient skeletal muscles. Transgenic over-expression of PGC-1α has been shown to increase levels of utrophin mRNA and improve the histology of mdx muscles.
Joshua T Selsby +4 more
doaj +8 more sources
Marginal level dystrophin expression improves clinical outcome in a strain of dystrophin/utrophin double knockout mice. [PDF]
PLoS ONE, 2010 Inactivation of all utrophin isoforms in dystrophin-deficient mdx mice results in a strain of utrophin knockout mdx (uko/mdx) mice. Uko/mdx mice display severe clinical symptoms and die prematurely as in Duchenne muscular dystrophy (DMD) patients.
Dejia Li, Yongping Yue, Dongsheng Duan
doaj +6 more sources
Utrophin up-regulation by an artificial transcription factor in transgenic mice. [PDF]
PLoS ONE, 2007 Duchenne Muscular Dystrophy (DMD) is a severe muscle degenerative disease, due to absence of dystrophin. There is currently no effective treatment for DMD.
Elisabetta Mattei +13 more
doaj +5 more sources
Distinct regions in the 3′ untranslated region are responsible for targeting and stabilizing utrophin transcripts in skeletal muscle cells [PDF]
, 2001 In this study, we have sought to determine whether utrophin transcripts are targeted to a distinct subcellular compartment in skeletal muscle cells, and have examined the role of the 3′ untranslated region (UTR) in regulating the stability and ...
Anthony O. Gramolini +2 more
openalex +4 more sources
Upregulation of utrophin improves the phenotype of Duchenne muscular dystrophy hiPSC-derived CMs. [PDF]
Mol Ther Nucleic AcidsDuchenne muscular dystrophy (DMD) is a genetic neuromuscular disease. Although it leads to muscle weakness, affected individuals predominantly die from cardiomyopathy, which remains uncurable.
Andrysiak K +14 more
europepmc +3 more sources
Hepatology, EarlyView., 2022 Adaptive mitochondrial mechanisms allow mitochondrial resilience and prevent the worsening of fibrosis, while deregulation of these mechanisms promotes the progression from no/minimal‐mild (F0‐F2) fibrosis to advanced fibrosis and cirrhosis (F3‐F4). Abstract Background and Aims Hepatitis B virus (HBV) infection causes oxidative stress (OS) and alters ...
Dimitri Loureiro +17 more
wiley +1 more source
PROTEOMICS, Volume 22, Issue 23-24, December 2022., 2022 Abstract The X‐linked inherited neuromuscular disorder Duchenne muscular dystrophy is characterised by primary abnormalities in the membrane cytoskeletal component dystrophin. The almost complete absence of the Dp427‐M isoform of dystrophin in skeletal muscles renders contractile fibres more susceptible to progressive degeneration and a leaky ...
Paul Dowling +3 more
wiley +1 more source