A5079688063- Muscle Physiology and Disorders
- GDF15 and Related Biomarkers
- RNA Research and Splicing
- Tissue Engineering and Regenerative Medicine
- Telomeres, Telomerase, and Senescence
- Pluripotent Stem Cells Research
- Connective tissue disorders research
- Adenosine and Purinergic Signaling
- Genetics, Aging, and Longevity in Model Organisms
University of Portsmouth
2019-2022
Abstract Background Duchenne muscular dystrophy (DMD) causes severe disability of children and death young men, with an incidence approximately 1/5000 male births. Symptoms appear in early childhood, a diagnosis made mostly around 4 years old, time where the amount muscle damage is already significant, preventing therapeutic interventions that could be more efficient at halting disease progression. In meantime, precise moment which phenotypes arise—even asymptomatically—is still unknown....
Duchenne muscular dystrophy (DMD) affects myofibers and muscle stem cells, causing progressive degeneration repair defects. It was unknown whether dystrophic myoblasts—the effector cells of growth regeneration—are affected. Using transcriptomic, genome-scale metabolic modelling functional analyses, we demonstrate, for the first time, convergent abnormalities in primary mouse human myoblasts. In Dmd mdx myoblasts lacking full-length dystrophin, expression 170 genes significantly altered....
Abstract Duchenne muscular dystrophy (DMD) affects myofibers and muscle stem cells (SC), causing progressive degeneration repair defects. It was not known whether dystrophic myoblasts—the effector of growth regeneration—are affected. Using a combination transcriptomic, molecular, functional analyses, genome-scale metabolic modelling, we demonstrate, for the first time, convergent cell-autonomous abnormalities in primary mouse human myoblasts. In Dmd mdx myoblasts lacking full-length...