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The MuSK-BMP pathway maintains myofiber size in slow muscle through regulation of Akt-mTOR signaling
Springer Science and Business Media LLC - - 2024
Myofiber size regulation is critical in health, disease, and aging. MuSK (muscle-specific kinase) is a BMP (bone morphogenetic protein) co-receptor that promotes and shapes BMP signaling. MuSK is expressed at all neuromuscular junctions and is also present extrasynaptically in the mouse soleus, whose predominantly oxidative fiber composition is akin to that of human muscle. To investigate the role...... hiện toàn bộ
Attenuated Ca2+ release in a mouse model of limb girdle muscular dystrophy 2A
Springer Science and Business Media LLC - Tập 6 - Trang 1-15 - 2016
Mutations in CAPN3 cause limb girdle muscular dystrophy type 2A (LGMD2A), a progressive muscle wasting disease. CAPN3 is a non-lysosomal, Ca-dependent, muscle-specific proteinase. Ablation of CAPN3 (calpain-3 knockout (C3KO) mice) leads to reduced ryanodine receptor (RyR1) expression and abnormal Ca2+/calmodulin-dependent protein kinase II (Ca-CaMKII)-mediated signaling. We previously reported tha...... hiện toàn bộ
Impaired regeneration in calpain-3 null muscle is associated with perturbations in mTORC1 signaling and defective mitochondrial biogenesis
Springer Science and Business Media LLC - Tập 7 - Trang 1-18 - 2017
Previous studies in patients with limb-girdle muscular dystrophy type 2A (LGMD2A) have suggested that calpain-3 (CAPN3) mutations result in aberrant regeneration in muscle. To gain insight into pathogenesis of aberrant muscle regeneration in LGMD2A, we used a paradigm of cardiotoxin (CTX)-induced cycles of muscle necrosis and regeneration in the CAPN3-KO mice to simulate the early features of the ...... hiện toàn bộ
An RNAi based screen in Drosophila larvae identifies fascin as a regulator of myoblast fusion and myotendinous junction structure
Springer Science and Business Media LLC - Tập 8 - Trang 1-13 - 2018
A strength of Drosophila as a model system is its utility as a tool to screen for novel regulators of various functional and developmental processes. However, the utility of Drosophila as a screening tool is dependent on the speed and simplicity of the assay used. Here, we use larval locomotion as an assay to identify novel regulators of skeletal muscle function. We combined this assay with muscle...... hiện toàn bộ
Dynamics of myogenic differentiation using a novel Myogenin knock-in reporter mouse
Springer Science and Business Media LLC - Tập 11 - Trang 1-13 - 2021
Myogenin is a transcription factor that is expressed during terminal myoblast differentiation in embryonic development and adult muscle regeneration. Investigation of this cell state transition has been hampered by the lack of a sensitive reporter to dynamically track cells during differentiation. Here, we report a knock-in mouse line expressing the tdTOMATO fluorescent protein from the endogenous...... hiện toàn bộ
The Notch signaling network in muscle stem cells during development, homeostasis, and diseaseAbstract Skeletal muscle stem cells have a central role in muscle growth and regeneration. They reside as quiescent cells in resting muscle and in response to damage they transiently amplify and fuse to produce new myofibers or self-renew to replenish the stem cell pool. A signaling pathway that is critical in the regulation of all these processes is Notch. Despite ... ... hiện toàn bộ
Springer Science and Business Media LLC - Tập 12 Số 1 - 2022
Skeletal muscle transcriptomics identifies common pathways in nerve crush injury and ageing
Springer Science and Business Media LLC - Tập 12 - Trang 1-20 - 2022
Motor unit remodelling involving repeated denervation and re-innervation occurs throughout life. The efficiency of this process declines with age contributing to neuromuscular deficits. This study investigated differentially expressed genes (DEG) in muscle following peroneal nerve crush to model motor unit remodelling in C57BL/6 J mice. Muscle RNA was isolated at 3 days post-crush, RNA libraries w...... hiện toàn bộ
MG53′s new identity
Springer Science and Business Media LLC - Tập 3 - Trang 1-3 - 2013
Mitsugumin 53 (MG53) is a relatively newly identified tripartite motif-containing (TRIM) family muscle-specific E3 ubiquitin ligase that is expressed in skeletal muscle and the heart. It has been postulated to facilitate repair by targeting the site of an injury, and acting as a scaffold for assembly of a repair complex made up of dysferlin, annexin V, caveolin-3, and polymerase I and transcript r...... hiện toàn bộ
ViaFuse: Fiji macros to calculate skeletal muscle cell viability and fusion index
Springer Science and Business Media LLC - Tập 11 - Trang 1-13 - 2021
Measuring biological features of skeletal muscle cells is difficult because of their unique morphology and multinucleate nature upon differentiation. Here, we developed a new Fiji macro package called ViaFuse (that stands for viability and fusion) to measure skeletal muscle cell viability and differentiation. To test ViaFuse, we utilized immunofluorescence images of differentiated myotubes where t...... hiện toàn bộ
DNA-binding sequence specificity of DUX4
Springer Science and Business Media LLC - Tập 6 - Trang 1-11 - 2016
Misexpression of the double homeodomain transcription factor DUX4 results in facioscapulohumeral muscular dystrophy (FSHD). A DNA-binding consensus with two tandem TAAT motifs based on chromatin IP peaks has been discovered; however, the consensus has multiple variations (flavors) of unknown relative activity. In addition, not all peaks have this consensus, and the Pitx1 promoter, the first DUX4 t...... hiện toàn bộ
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