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Skeletal Muscle

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https://www.readbyqxmd.com/read/30368256/a-novel-tetracycline-responsive-transgenic-mouse-strain-for-skeletal-muscle-specific-gene-expression
#1
Masahiro Iwata, Davis A Englund, Yuan Wen, Cory M Dungan, Kevin A Murach, Ivan J Vechetti, Christopher B Mobley, Charlotte A Peterson, John J McCarthy
BACKGROUND: The tetracycline-responsive system (Tet-ON/OFF) has proven to be a valuable tool for manipulating gene expression in an inducible, temporal, and tissue-specific manner. The purpose of this study was to create and characterize a new transgenic mouse strain utilizing the human skeletal muscle α-actin (HSA) promoter to drive skeletal muscle-specific expression of the reverse tetracycline transactivator (rtTA) gene which we have designated as the HSA-rtTA mouse. METHODS: To confirm the HSA-rtTA mouse was capable of driving skeletal muscle-specific expression, we crossed the HSA-rtTA mouse with the tetracycline-responsive histone H2B-green fluorescent protein (H2B-GFP) transgenic mouse in order to label myonuclei...
October 27, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30368252/myostatin-and-activin-blockade-by-engineered-follistatin-results-in-hypertrophy-and-improves-dystrophic-pathology-in-mdx-mouse-more-than-myostatin-blockade-alone
#2
Andrea Iskenderian, Nan Liu, Qingwei Deng, Yan Huang, Chuan Shen, Kathleen Palmieri, Robert Crooker, Dianna Lundberg, Niksa Kastrapeli, Brian Pescatore, Alla Romashko, John Dumas, Robert Comeau, Angela Norton, Jing Pan, Haojing Rong, Katayoun Derakhchan, David E Ehmann
BACKGROUND: Myostatin antagonists are being developed as therapies for Duchenne muscular dystrophy due to their strong hypertrophic effects on skeletal muscle. Engineered follistatin has the potential to combine the hypertrophy of myostatin antagonism with the anti-inflammatory and anti-fibrotic effects of activin A antagonism. METHODS: Engineered follistatin was administered to C57BL/6 mice for 4 weeks, and muscle mass and myofiber size was measured. In the mdx model, engineered follistatin was dosed for 12 weeks in two studies comparing to an Fc fusion of the activin IIB receptor or an anti-myostatin antibody...
October 27, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30336774/automated-muscle-histopathology-analysis-using-cellprofiler
#3
Yeh Siang Lau, Li Xu, Yandi Gao, Renzhi Han
BACKGROUND: Histological assessment of skeletal muscle sections is important for the research of muscle physiology and diseases. Quantifiable measures of skeletal muscle often include mean fiber diameter, fiber size distribution, and centrally nucleated muscle fibers. These parameters offer insights into the dynamic adaptation of skeletal muscle cells during repeated cycles of degeneration and regeneration associated with many muscle diseases and injuries. Computational programs designed to obtain these parameters would greatly facilitate such efforts and offer significant advantage over manual image analysis, which is very labor-intensive and often subjective...
October 18, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30305165/muscle-membrane-integrity-in-duchenne-muscular-dystrophy-recent-advances-in-copolymer-based-muscle-membrane-stabilizers
#4
REVIEW
Evelyne M Houang, Yuk Y Sham, Frank S Bates, Joseph M Metzger
The scientific premise, design, and structure-function analysis of chemical-based muscle membrane stabilizing block copolymers are reviewed here for applications in striated muscle membrane injury. Synthetic block copolymers have a rich history and wide array of applications from industry to biology. Potential for discovery is enabled by a large chemical space for block copolymers, including modifications in block copolymer mass, composition, and molecular architecture. Collectively, this presents an impressive chemical landscape to leverage distinct structure-function outcomes...
October 10, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30231928/deletion-of-the-microtubule-associated-protein-6-map6-results-in-skeletal-muscle-dysfunction
#5
Muriel Sébastien, Benoit Giannesini, Perrine Aubin, Julie Brocard, Mathilde Chivet, Laura Pietrangelo, Simona Boncompagni, Christophe Bosc, Jacques Brocard, John Rendu, Sylvie Gory-Fauré, Annie Andrieux, Anne Fourest-Lieuvin, Julien Fauré, Isabelle Marty
BACKGROUND: The skeletal muscle fiber has a specific and precise intracellular organization which is at the basis of an efficient muscle contraction. Microtubules are long known to play a major role in the function and organization of many cells, but in skeletal muscle, the contribution of the microtubule cytoskeleton to the efficiency of contraction has only recently been studied. The microtubule network is dynamic and is regulated by many microtubule-associated proteins (MAPs). In the present study, the role of the MAP6 protein in skeletal muscle organization and function has been studied using the MAP6 knockout mouse line...
September 19, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30219099/the-effects-of-neuregulin-1%C3%AE-on-intrafusal-muscle-fiber-formation-in-neuromuscular-coculture-of-dorsal-root-ganglion-explants-and-skeletal-muscle-cells
#6
Yuan Qiao, Menglin Cong, Jianmin Li, Hao Li, Zhenzhong Li
BACKGROUND: The formation of intrafusal muscle (IM) fibers and their contact with afferent proprioceptive axons is critical for construction, function, and maintenance of the stretch reflex. Many factors affect the formation of IM fibers. Finding new factors and mechanisms of IM fiber formation is essential for the reconstruction of stretch reflex arc after injury. METHODS: We established a coculture system of organotypic dorsal root ganglion (DRG) explants and dissociated skeletal muscle (SKM) cells...
September 15, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30153853/biochemical-and-pathological-changes-result-from-mutated-caveolin-3-in-muscle
#7
José Andrés González Coraspe, Joachim Weis, Mary E Anderson, Ute Münchberg, Kristina Lorenz, Stephan Buchkremer, Stephanie Carr, René Peiman Zahedi, Eva Brauers, Hannah Michels, Yoshihide Sunada, Hanns Lochmüller, Kevin P Campbell, Erik Freier, Denisa Hathazi, Andreas Roos
BACKGROUND: Caveolin-3 (CAV3) is a muscle-specific protein localized to the sarcolemma. It was suggested that CAV3 is involved in the connection between the extracellular matrix (ECM) and the cytoskeleton. Caveolinopathies often go along with increased CK levels indicative of sarcolemmal damage. So far, more than 40 dominant pathogenic mutations have been described leading to several phenotypes many of which are associated with a mis-localization of the mutant protein to the Golgi. Golgi retention and endoplasmic reticulum (ER) stress has been demonstrated for the CAV3 p...
August 28, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30139390/visualization-of-pax7-protein-dynamics-in-muscle-satellite-cells-in-a-yfp-knock-in-mouse-line
#8
Yasuo Kitajima, Yusuke Ono
BACKGROUND: Satellite cells are residential muscle stem cells that express a paired box protein, PAX7. RESULTS: Here, we report a knock-in mouse line expressing a PAX7-enhanced yellow fluorescent protein (YFP) fusion protein that enables visualization of PAX7 protein dynamics in living satellite cells through YFP fluorescence. The YFP fluorescence signals in Pax7-YFP knock-in mice clearly recapitulated the endogenous expression of PAX7 protein in satellite cells...
August 24, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30139374/a-robust-pax7egfp-mouse-that-enables-the-visualization-of-dynamic-behaviors-of-muscle-stem-cells
#9
Elisia D Tichy, David K Sidibe, Christopher D Greer, Nicholas M Oyster, Panteleimon Rompolas, Nadia A Rosenthal, Helen M Blau, Foteini Mourkioti
BACKGROUND: Pax7 is a transcription factor involved in the specification and maintenance of muscle stem cells (MuSCs). Upon injury, MuSCs leave their quiescent state, downregulate Pax7 and differentiate, contributing to skeletal muscle regeneration. In the majority of regeneration studies, MuSCs are isolated by fluorescence-activated sorting (FACS), based on cell surface markers. It is known that MuSCs are a heterogeneous population and only a small percentage of isolated cells are true stem cells that are able to self-renew...
August 24, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30081940/musclej-a-high-content-analysis-method-to-study-skeletal-muscle-with-a-new-fiji-tool
#10
Alicia Mayeuf-Louchart, David Hardy, Quentin Thorel, Pascal Roux, Lorna Gueniot, David Briand, Aurélien Mazeraud, Adrien Bouglé, Spencer L Shorte, Bart Staels, Fabrice Chrétien, Hélène Duez, Anne Danckaert
BACKGROUND: Skeletal muscle has the capacity to adapt to environmental changes and regenerate upon injury. To study these processes, most experimental methods use quantification of parameters obtained from images of immunostained skeletal muscle. Muscle cross-sectional area, fiber typing, localization of nuclei within the muscle fiber, the number of vessels, and fiber-associated stem cells are used to assess muscle physiology. Manual quantification of these parameters is time consuming and only poorly reproducible...
August 6, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30071896/identification-of-smchd1-domains-for-nuclear-localization-homo-dimerization-and-protein-cleavage
#11
Yosuke Hiramuki, Stephen J Tapscott
BACKGROUND: SMCHD1 is a disease modifier and a causative gene for facioscapulohumeral muscular dystrophy (FSHD) type 1 and type 2, respectively. A large variety of different mutations in SMCHD1 have been identified as causing FSHD2. In many cases, it is unclear how these mutations disrupt the normal function of SMCHD1. METHODS: We made and analyzed lenti-viral vectors that express Flag-tagged full-length or different mutant SMCHD1 proteins to better understand the functional domains of SMCHD1 in muscle cells...
August 2, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30060766/detection-of-variants-in-dystroglycanopathy-associated-genes-through-the-application-of-targeted-whole-exome-sequencing-analysis-to-a-large-cohort-of-patients-with-unexplained-limb-girdle-muscle-weakness
#12
MULTICENTER STUDY
Katherine Johnson, Marta Bertoli, Lauren Phillips, Ana Töpf, Peter Van den Bergh, John Vissing, Nanna Witting, Shahriar Nafissi, Shirin Jamal-Omidi, Anna Łusakowska, Anna Kostera-Pruszczyk, Anna Potulska-Chromik, Nicolas Deconinck, Carina Wallgren-Pettersson, Sonja Strang-Karlsson, Jaume Colomer, Kristl G Claeys, Willem De Ridder, Jonathan Baets, Maja von der Hagen, Roberto Fernández-Torrón, Miren Zulaica Ijurco, Juan Bautista Espinal Valencia, Andreas Hahn, Hacer Durmus, Tracey Willis, Liwen Xu, Elise Valkanas, Thomas E Mullen, Monkol Lek, Daniel G MacArthur, Volker Straub
BACKGROUND: Dystroglycanopathies are a clinically and genetically heterogeneous group of disorders that are typically characterised by limb-girdle muscle weakness. Mutations in 18 different genes have been associated with dystroglycanopathies, the encoded proteins of which typically modulate the binding of α-dystroglycan to extracellular matrix ligands by altering its glycosylation. This results in a disruption of the structural integrity of the myocyte, ultimately leading to muscle degeneration...
July 30, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30025545/voltage-sensing-mechanism-in-skeletal-muscle-excitation-contraction-coupling-coming-of-age-or-midlife-crisis
#13
REVIEW
Erick O Hernández-Ochoa, Martin F Schneider
The process by which muscle fiber electrical depolarization is linked to activation of muscle contraction is known as excitation-contraction coupling (ECC). Our understanding of ECC has increased enormously since the early scientific descriptions of the phenomenon of electrical activation of muscle contraction by Galvani that date back to the end of the eighteenth century. Major advances in electrical and optical measurements, including muscle fiber voltage clamp to reveal membrane electrical properties, in conjunction with the development of electron microscopy to unveil structural details provided an elegant view of ECC in skeletal muscle during the last century...
July 19, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/30025544/whole-body-clearing-staining-and-screening-of-calcium-deposits-in-the-mdx-mouse-model-of-duchenne-muscular-dystrophy
#14
Lukasz Bozycki, Kacper Łukasiewicz, Paweł Matryba, Slawomir Pikula
BACKGROUND: Duchenne muscular dystrophy (DMD) is a fatal, X-linked genetic disorder. Although DMD is the most common form of muscular dystrophy, only two FDA-approved drugs were developed to delay its progression. In order to assess therapies for treating DMD, several murine models have recently been introduced. As the wide variety of murine models enlighten mechanisms underlying DMD pathology, the question on how to monitor the progression of the disease within the entire musculoskeletal system still remains to be answered...
July 19, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29973273/klotho-expression-is-a-prerequisite-for-proper-muscle-stem-cell-function-and-regeneration-of-skeletal-muscle
#15
Hellen E Ahrens, Judith Huettemeister, Manuel Schmidt, Christoph Kaether, Julia von Maltzahn
BACKGROUND: Klotho is a well-known anti-aging hormone, which serves as a suppressor of aging through a variety of mechanisms. Aging of skeletal muscle is concomitant with a decrease in muscle stem cell function resulting in impaired regeneration. METHODS: Here we investigate the functional role of the anti-aging hormone Klotho for muscle stem cell function after cardiotoxin-induced injury of skeletal muscle using a klotho hypomorphic mouse line, which is characterized by a premature aging phenotype...
July 4, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29875011/correction-to-comparison-of-multiple-transcriptomes-exposes-unified-and-divergent-features-of-quiescent-and-activated-skeletal-muscle-stem-cells
#16
Natalia Pietrosemoli, Sébastien Mella, Siham Yennek, Meryem B Baghdadi, Hiroshi Sakai, Ramkumar Sambasivan, Francesca Pala, Daniela Di Girolamo, Shahragim Tajbakhsh
After publication of this article [1], the authors noted that the legends for supplementary files Figures S3 and S4 were truncated in the production process, therefore lacking some information concerning these Figures. The complete legends are included in this Correction. The authors apologize for any inconvenience that this might have caused.
June 6, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29855350/dietary-supplementation-with-ketoacids-protects-against-ckd-induced-oxidative-damage-and-mitochondrial-dysfunction-in-skeletal-muscle-of-5-6-nephrectomised-rats
#17
Dongtao Wang, Lianbo Wei, Yajun Yang, Huan Liu
BACKGROUND: A low-protein diet supplemented with ketoacids (LPD + KA) maintains the nutritional status of patients with chronic kidney disease (CKD). Oxidative damage and mitochondrial dysfunction associated with the upregulation of p66SHC and FoxO3a have been shown to contribute to muscle atrophy. This study aimed to determine whether LPD + KA improves muscle atrophy and attenuates the oxidative stress and mitochondrial damage observed in CKD rats. METHODS: 5/6 nephrectomy rats were randomly divided into three groups and fed with either 22% protein (normal-protein diet; NPD), 6% protein (low-protein diets; LPD) or 5% protein plus 1% ketoacids (LPD + KA) for 24 weeks...
May 31, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29855340/trappc11-and-gosr2-mutations-associate-with-hypoglycosylation-of-%C3%AE-dystroglycan-and-muscular-dystrophy
#18
Austin A Larson, Peter R Baker, Miroslav P Milev, Craig A Press, Ronald J Sokol, Mary O Cox, Jacqueline K Lekostaj, Aaron A Stence, Aaron D Bossler, Jennifer M Mueller, Keshika Prematilake, Thierry Fotsing Tadjo, Charles A Williams, Michael Sacher, Steven A Moore
BACKGROUND: Transport protein particle (TRAPP) is a supramolecular protein complex that functions in localizing proteins to the Golgi compartment. The TRAPPC11 subunit has been implicated in muscle disease by virtue of homozygous and compound heterozygous deleterious mutations being identified in individuals with limb girdle muscular dystrophy and congenital muscular dystrophy. It remains unclear how this protein leads to muscle disease. Furthermore, a role for this protein, or any other membrane trafficking protein, in the etiology of the dystroglycanopathy group of muscular dystrophies has yet to be found...
May 31, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29843823/a-novel-canine-model-for-duchenne-muscular-dystrophy-dmd-single-nucleotide-deletion-in-dmd-gene-exon-20
#19
Sara Mata López, James J Hammond, Madison B Rigsby, Cynthia J Balog-Alvarez, Joe N Kornegay, Peter P Nghiem
BACKGROUND: Boys with Duchenne muscular dystrophy (DMD) have DMD gene mutations, with associated loss of the dystrophin protein and progressive muscle degeneration and weakness. Corticosteroids and palliative support are currently the best treatment options. The long-term benefits of recently approved compounds such as eteplirsen and ataluren remain to be seen. Dogs with naturally occurring dystrophinopathies show progressive disease akin to that of DMD. Accordingly, canine DMD models are useful for studies of pathogenesis and preclinical therapy development...
May 29, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29703249/mir-708-5p-and-mir-34c-5p-are-involved-in-nnos-regulation-in-dystrophic-context
#20
Marine Guilbaud, Christel Gentil, Cécile Peccate, Elena Gargaun, Isabelle Holtzmann, Carole Gruszczynski, Sestina Falcone, Kamel Mamchaoui, Rabah Ben Yaou, France Leturcq, Laurence Jeanson-Leh, France Piétri-Rouxel
BACKGROUND: Duchenne (DMD) and Becker (BMD) muscular dystrophies are caused by mutations in the DMD gene coding for dystrophin, a protein being part of a large sarcolemmal protein scaffold that includes the neuronal nitric oxide synthase (nNOS). The nNOS was shown to play critical roles in a variety of muscle functions and alterations of its expression and location in dystrophic muscle fiber leads to an increase of the muscle fatigability. We previously revealed a decrease of nNOS expression in BMD patients all presenting a deletion of exons 45 to 55 in the DMD gene (BMDd45-55), impacting the nNOS binding site of dystrophin...
April 27, 2018: Skeletal Muscle
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