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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
Clothilde Claus, Moriya Slavin, Eugénie Ansseau, Céline Lancelot, Karimatou Bah, Saskia Lassche, Manon Fiévet, Anna Greco, Sara Tomaiuolo, Alexandra Tassin, Virginie Dudome, Benno Kusters, Anne-Emilie Declèves, Dalila Laoudj-Chenivesse, Baziel G. M. van Engelen, Denis Nonclercq, Alexandra Belayew, Nir Kalisman, Frédérique Coppée
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 (...... hiện toàn bộ
Skeletal muscle interleukin 15 promotes CD8+ T-cell function and autoimmune myositis
Springer Science and Business Media LLC - Tập 5 - Trang 1-14 - 2015
Po-Lin Huang, Mau-Sheng Hou, Szu-Wen Wang, Chin-Ling Chang, Yae-Huei Liou, Nan-Shih Liao
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....... hiện toàn bộ
Muscle spindle function in healthy and diseased muscle
Springer Science and Business Media LLC - Tập 11 - Trang 1-13 - 2021
Stephan Kröger, Bridgette Watkins
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...... hiện toàn bộ
Functional effects of muscle PGC-1alpha in aged animals
Springer Science and Business Media LLC - Tập 10 Số 1 - 2020
Steven Yang, Emanuele Loro, Shogo Wada, Boa Kim, Wei‐Ju Tseng, Kristina Li, Tejvir S. Khurana, Zoltàn Arany
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, l...... hiện toàn bộ
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
Yuan Qiao, Menglin Cong, Jianmin Li, Hao Li, Zhenzhong Li
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
Stijn L. M. in ‘t Groen, Marnix Franken, Theresa Bock, Marcus Krüger, Jessica C. de Greef, W. W. M. Pim Pijnappel
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...... hiện toàn bộ
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
Angus Lindsay, Cory W. Baumann, Robyn T. Rebbeck, Samantha L. Yuen, William M. Southern, James S. Hodges, Razvan L. Cornea, David D. Thomas, James M. Ervasti, Dawn A. Lowe
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%), ...... hiện toàn bộ
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
Regan-Heng Zhang, Robert N. Judson, David Y. Liu, Jürgen Kast, Fabio M. V. Rossi
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...... hiện toàn bộ
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
Haitao A. Wang, John D. Lee, Kah Meng Lee, Trent M. Woodruff, Peter G. Noakes
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...... hiện toàn bộ
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
Fabrizio Rinaldi, Yu Zhang, Ricardo Mondragon-Gonzalez, Jeffrey Harvey, Rita C. R. Perlingeiro
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 ...... hiện toàn bộ
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