Skeletal muscle‐specific PGC‐1α‐b overexpression prevents eccentric contraction‐induced muscle injury through an utrophin‐independent pathway in mice [PDF]
Physiological ReportsSlower oxidative fibers are more resistant to eccentric contraction (ECC)‐induced muscle damage than fast‐twitch glycolytic fibers, but the mechanisms remain unclear. This study investigated the roles of the exercise‐inducible PGC‐1α isoform PGC‐1α‐b and
Azuma Naito +7 more
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Systemic MyoAAV-saRNA delivery activates endogenous utrophin and rescues dystrophic pathology in mdx mice [PDF]
Regenerative TherapyBackground: Therapeutic activation of endogenous utrophin, a homolog of dystrophin, represents a mutation-agnostic therapeutic strategy with considerable potential for treating Duchenne muscular dystrophy (DMD).
Zhikai Xiahou +8 more
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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 +5 more sources
Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice. [PDF]
PLoS ONE, 2011 Duchenne muscular dystrophy (DMD) is an incurable degenerative muscle disorder. We injected WT mouse induced pluripotent stem cells (iPSCs) into mdx and mdx∶utrophin mutant blastocysts, which are predisposed to develop DMD with an increasing degree of ...
Amanda J Beck +9 more
doaj +3 more sources
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
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Molecular Metabolism, 2021 Objectives: Preferential damage to fast, glycolytic myofibers is common in many muscle-wasting diseases, including Duchenne muscular dystrophy (DMD).
Justin P. Hardee +16 more
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Activation of endogenous full-length utrophin by MyoAAV-UA as a therapeutic approach for Duchenne muscular dystrophy [PDF]
Nature CommunicationsActivation of endogenous full-length utrophin, a dystrophin homolog, presents an attractive therapeutic strategy for Duchenne muscular dystrophy (DMD), regardless of mutation types and loci.
Ruo Wu +18 more
doaj +2 more sources
L-Arginine Activates the Neuregulin-1/ErbB Receptor Signaling Pathway and Increases Utrophin mRNA Levels in C2C12 Cells [PDF]
Biochemistry Research InternationalL-arginine induces the expression of utrophin in skeletal muscle cells, so it has been proposed as a pharmacological treatment to attenuate the symptoms of Duchenne muscular dystrophy (DMD).
Gladys Tapia +6 more
doaj +2 more sources
Dystrophin Restorative and Compensatory Gene Addition Therapies for Duchenne Muscular Dystrophy: Could CRISPRa Provide a Realistic Alternative? [PDF]
MusclesDuchenne muscular dystrophy (DMD), which results from mutations that disrupt the expression of dystrophin proteins, is characterized by progressive muscle fiber wasting and the development of skeletal muscle fibrosis.
Zakaria Rostamitehrani +2 more
doaj +2 more sources
Biallelic variants in the UTRN gene cause a novel form of multiple congenital arthrogryposis [PDF]
Frontiers in GeneticsArthrogryposis multiplex congenita (AMC) is a large group of congenital conditions characterized by joint contractures affecting two or more body areas.
Evgeniya Melnik +9 more
doaj +2 more sources