Muscle spindle function in healthy and diseased muscleSpringer 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
The lysine methyltransferase Ehmt2/G9a is dispensable for skeletal muscle development and regenerationSpringer 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
Treatment with rGDF11 does not improve the dystrophic muscle pathology of mdx miceSpringer 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
High-throughput screening identifies inhibitors of DUX4-induced myoblast toxicitySpringer Science and Business Media LLC - Tập 4 - Trang 1-11 - 2014
Darko Bosnakovski, Si Ho Choi, Jessica M Strasser, Erik A Toso, Michael A Walters, Michael Kyba
Facioscapulohumeral muscular dystrophy (FSHD) is caused by epigenetic alterations at the D4Z4 macrosatellite repeat locus on chromosome 4, resulting in inappropriate expression of the DUX4 protein. The DUX4 protein is therefore the primary molecular target for therapeutic intervention. We have developed a high-throughput screen based on the toxicity of DUX4 when overexpressed in C2C12 myoblasts, a...... hiện toàn bộ
Heme oxygenase and carbon monoxide protect from muscle dystrophySpringer Science and Business Media LLC - Tập 6 - Trang 1-12 - 2016
Mun Chun Chan, Olivia Ziegler, Laura Liu, Glenn C. Rowe, Saumya Das, Leo E. Otterbein, Zoltan Arany
Duchenne muscle dystrophy (DMD) is one of the most common lethal genetic diseases of children worldwide and is 100% fatal. Steroids, the only therapy currently available, are marred by poor efficacy and a high side-effect profile. New therapeutic approaches are urgently needed. Here, we leverage PGC-1α, a powerful transcriptional coactivator known to protect against dystrophy in the mdx murine mod...... hiện toàn bộ
PPARδ regulates satellite cell proliferation and skeletal muscle regenerationSpringer Science and Business Media LLC - Tập 1 Số 1 - 2011
Alison Rae Angione, Chunhui Jiang, Dongning Pan, Yong‐Xu Wang, Shihuan Kuang
Abstract
Peroxisome proliferator-activated receptors (PPARs) are a class of nuclear receptors that play important roles in development and energy metabolism. Whereas PPARδ has been shown to regulate mitochondrial biosynthesis and slow-muscle fiber types, its function in skeletal muscle progenitors (satellite cells) is unknown. Since constitutive mutation ...... hiện toàn bộ
A human skeletal muscle interactome centered on proteins involved in muscular dystrophies: LGMD interactomeSpringer Science and Business Media LLC - Tập 3 - Trang 1-19 - 2013
Gaëlle Blandin, Sylvie Marchand, Karine Charton, Nathalie Danièle, Evelyne Gicquel, Jean-Baptiste Boucheteil, Azéddine Bentaib, Laetitia Barrault, Daniel Stockholm, Marc Bartoli, Isabelle Richard
The complexity of the skeletal muscle and the identification of numerous human disease-causing mutations in its constitutive proteins make it an interesting tissue for proteomic studies aimed at understanding functional relationships of interacting proteins in both health and diseases. We undertook a large-scale study using two-hybrid screens and a human skeletal-muscle cDNA library to establish a...... hiện toàn bộ
Skeletal muscle characteristics are preserved in hTERT/cdk4 human myogenic cell linesSpringer Science and Business Media LLC - Tập 6 - Trang 1-12 - 2016
Matthew Thorley, Stéphanie Duguez, Emilia Maria Cristina Mazza, Sara Valsoni, Anne Bigot, Kamel Mamchaoui, Brennan Harmon, Thomas Voit, Vincent Mouly, William Duddy
hTERT/cdk4 immortalized myogenic human cell lines represent an important tool for skeletal muscle research, being used as therapeutically pertinent models of various neuromuscular disorders and in numerous fundamental studies of muscle cell function. However, the cell cycle is linked to other cellular processes such as integrin regulation, the PI3K/Akt pathway, and microtubule stability, raising t...... hiện toàn bộ