Results 21 to 30 of about 7,866 (244)
Fatigue Resistance and Mitochondrial Adaptations to Isometric Interval Training in Dystrophin-Deficient Muscle: Role of Contractile Load. [PDF]
FASEB JHigh‐load, but not low‐load, isometric interval training (IT) increased mitochondrial content and muscle fatigue resistance in mdx52 mice, a model for Duchenne muscular dystrophy. High‐load isometric IT restored citrate synthase activity, peroxisome proliferator‐activated receptor γ coactivator 1 alpha (PGC‐1α) expression, and mitochondrial complex II ...
Yamauchi N +7 more
europepmc +2 more sources
Genome Editing-Mediated Utrophin Upregulation in Duchenne Muscular Dystrophy Stem Cells
Molecular Therapy: Nucleic Acids, 2020 Utrophin upregulation is considered a promising therapeutic strategy for Duchenne muscular dystrophy (DMD). A number of microRNAs (miRNAs) post-transcriptionally regulate utrophin expression by binding their cognate sites in the 3′ UTR.
Kasturi Sengupta +5 more
doaj +1 more source
FASEB BioAdvances, 2021 Duchenne muscular dystrophy (DMD) is a genetic disorder that results in the absence of dystrophin, a cytoskeletal protein. Individuals with this disease experience progressive muscle destruction, which leads to muscle weakness.
Roy W. R. Dudley +3 more
doaj +1 more source
Molecular and Functional Analysis of the Utrophin Promoter [PDF]
Nucleic Acids Research, 1996 Utrophin is a ubiquitously expressed cytoskeletal protein which is an important structural component of the mammalian neuromuscular junction. It shows extensive sequence similarity to dystrophin leading to postulation that utrophin may be able to compensate for the absence of dystrophin in Duchenne muscular dystrophy (DMD) patients.
Cindi L. Dennis +3 more
openalex +3 more sources
An ex vivo gene therapy approach to treat muscular dystrophy using inducible pluripotent stem cells. [PDF]
, 2013 Duchenne muscular dystrophy is a progressive and incurable neuromuscular disease caused by genetic and biochemical defects of the dystrophin-glycoprotein complex.
Borges, Luciene +11 more
core +2 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 +1 more source
Functional characterization of orbicularis oculi and extraocular muscles [PDF]
, 2016 The orbicularis oculi are the sphincter muscles of the eyelids and are involved in modulating facial expression. They differ from both limb and extraocular muscles (EOMs) in their histology and biochemistry. Weakness of the orbicularis oculi muscles is a
Goldblum, David +5 more
core +2 more sources
Nature Communications, 2020 One potential approach for the treatment of Duchenne muscular dysrophy is to increase expression of the dystrophin homolog utrophin. Here, the authors show that eEF1A2 regulates utrophin expression, and show that 2 FDA-approved drugs upregulate eEIF1A2 ...
Christine Péladeau +10 more
doaj +1 more source
Review: Dystroglycan in the Nervous System [PDF]
, 2007 Dystroglycan is part of a large complex of proteins, the dystrophin-glycoprotein complex, which has been implicated in the pathogenesis of muscular dystrophies for a long time.
Matthias Samwald
core +2 more sources
Microtubule binding distinguishes dystrophin from utrophin [PDF]
Proceedings of the National Academy of Sciences, 2014 Significance Our in vitro analyses reveal that dystrophin, the protein absent in Duchenne muscular dystrophy patients, binds microtubules with high affinity and pauses microtubule polymerization, whereas utrophin, the autosomal homologue of dystrophin thought to mirror many known functions of dystrophin, has no activity in either assay.
Joseph J, Belanto +7 more
openaire +2 more sources