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The double homeodomain protein DUX4c is associated with regenerating muscle fibers and RNA-binding proteins
Springer Science and Business Media LLC - Tập 13 - Trang 1-30 - 2023
We have previously demonstrated that double homeobox 4 centromeric (DUX4C)
encoded for a functional DUX4c protein upregulated in dystrophic skeletal
muscles. Based on gain- and loss-of-function studies we have proposed DUX4c
involvement in muscle regeneration. Here, we provide further evidence for such a
role in skeletal muscles from patients affected with facioscapulohumeral
muscular dystrophy (...
Skeletal muscle interleukin 15 promotes CD8+ T-cell function and autoimmune myositis
Springer Science and Business Media LLC - Tập 5 - Trang 1-14 - 2015
Interleukin 15 (IL-15) is thought to be abundant in the skeletal muscle under
steady state conditions based on RNA expression; however, the IL-15 RNA level
may not reflect the protein level due to post-transcriptional regulation.
Although exogenous protein treatment and overexpression studies indicated IL-15
functions in the skeletal muscle, how the skeletal muscle cell uses IL-15
remains unclear....
Muscle spindle function in healthy and diseased muscle
Springer Science and Business Media LLC - Tập 11 - Trang 1-13 - 2021
Almost every muscle contains muscle spindles. These delicate sensory receptors
inform the central nervous system (CNS) about changes in the length of
individual muscles and the speed of stretching. With this information, the CNS
computes the position and movement of our extremities in space, which is a
requirement for motor control, for maintaining posture and for a stable gait.
Many neuromuscular...
Functional effects of muscle PGC-1alpha in aged animals
Springer Science and Business Media LLC - Tập 10 Số 1 - 2020
AbstractPGC-1 (peroxisome-proliferator-activated receptor-γ coactivator-1) alpha
is a potent transcriptional coactivator that coordinates the activation of
numerous metabolic processes. Exercise strongly induces PGC-1alpha expression in
muscle, and overexpression of PGC-1alpha in skeletal muscle activates
mitochondrial oxidative metabolism and neovascularization, leading to markedly
increased endu...
The effects of neuregulin-1β on intrafusal muscle fiber formation in neuromuscular coculture of dorsal root ganglion explants and skeletal muscle cells
Springer Science and Business Media LLC - - 2018
A knock down strategy for rapid, generic, and versatile modelling of muscular dystrophies in 3D-tissue-engineered-skeletal muscle
Springer Science and Business Media LLC - - 2024
Human iPSC-derived 3D-tissue-engineered-skeletal muscles (3D-TESMs) offer
advanced technology for disease modelling. However, due to the inherent genetic
heterogeneity among human individuals, it is often difficult to distinguish
disease-related readouts from random variability. The generation of genetically
matched isogenic controls using gene editing can reduce variability, but the
generation of...
Mechanical factors tune the sensitivity of mdx muscle to eccentric strength loss and its protection by antioxidant and calcium modulators
Springer Science and Business Media LLC - Tập 10 - Trang 1-14 - 2020
Dystrophin deficiency sensitizes skeletal muscle of mice to eccentric
contraction (ECC)-induced strength loss. ECC protocols distinguish
dystrophin-deficient from healthy, wild type muscle, and test the efficacy of
therapeutics for Duchenne muscular dystrophy (DMD). However, given the large
lab-to-lab variability in ECC-induced strength loss of dystrophin-deficient
mouse skeletal muscle (10–95%), ...
The lysine methyltransferase Ehmt2/G9a is dispensable for skeletal muscle development and regeneration
Springer Science and Business Media LLC - Tập 6 - Trang 1-10 - 2016
Euchromatic histone-lysine N-methyltransferase 2 (G9a/Ehmt2) is the main enzyme
responsible for the apposition of H3K9 di-methylation on histones. Due to its
dual role as an epigenetic regulator and in the regulation of non-histone
proteins through direct methylation, G9a has been implicated in a number of
biological processes relevant to cell fate control. Recent reports employing in
vitro cell l...
Complement C5a-C5aR1 signalling drives skeletal muscle macrophage recruitment in the hSOD1G93A mouse model of amyotrophic lateral sclerosis
Springer Science and Business Media LLC - Tập 7 - Trang 1-11 - 2017
The terminal pathway of the innate immune complement system is implicated in the
pathogenesis of amyotrophic lateral sclerosis (ALS). Terminal complement
activation leads to generation of C5a, which through its receptor, C5aR1, drives
immune cell recruitment and activation. Importantly, genetic or pharmacological
blockage of C5aR1 improves motor performance and reduces disease pathology in
hSOD1G9...
Treatment with rGDF11 does not improve the dystrophic muscle pathology of mdx mice
Springer Science and Business Media LLC - Tập 6 - Trang 1-8 - 2016
Duchenne muscular dystrophy (DMD) is an inherited lethal muscle wasting disease
characterized by cycles of degeneration and regeneration, with no effective
therapy. Growth differentiation factor 11 (GDF11), a member of the TGF-β
superfamily and myostatin homologous, has been reported to have the capacity to
reverse age-related skeletal muscle loss. These initial findings led us to
investigate the ...
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