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Skeletal Muscle

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https://www.readbyqxmd.com/read/29973273/klotho-expression-is-a-prerequisite-for-proper-muscle-stem-cell-function-and-regeneration-of-skeletal-muscle
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/29665848/characterization-and-utilization-of-the-flexor-digitorum-brevis-for-assessing-skeletal-muscle-function
#7
Michael D Tarpey, Adam J Amorese, Nicholas P Balestrieri, Terence E Ryan, Cameron A Schmidt, Joseph M McClung, Espen E Spangenburg
BACKGROUND: The ability to assess skeletal muscle function and delineate regulatory mechanisms is essential to uncovering therapeutic approaches that preserve functional independence in a disease state. Skeletal muscle provides distinct experimental challenges due to inherent differences across muscle groups, including fiber type and size that may limit experimental approaches. The flexor digitorum brevis (FDB) possesses numerous properties that offer the investigator a high degree of experimental flexibility to address specific hypotheses...
April 17, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29625624/an-rnai-based-screen-in-drosophila-larvae-identifies-fascin-as-a-regulator-of-myoblast-fusion-and-myotendinous-junction-structure
#8
Jaclyn M Camuglia, Torrey R Mandigo, Richard Moschella, Jenna Mark, Christine H Hudson, Derek Sheen, Eric S Folker
BACKGROUND: 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. METHODS: Here, we use larval locomotion as an assay to identify novel regulators of skeletal muscle function. We combined this assay with muscle-specific depletion of 82 genes to identify genes that impact muscle function by their expression in muscle cells...
April 6, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29625576/skeletal-cardiac-and-respiratory-muscle-function-and-histopathology-in-the-p448lneo-mouse-model-of-fkrp-deficient-muscular-dystrophy
#9
Qing Yu, Melissa Morales, Ning Li, Alexander G Fritz, Ren Ruobing, Anthony Blaeser, Ershia Francois, Qi-Long Lu, Kanneboyina Nagaraju, Christopher F Spurney
BACKGROUND: Fukutin-related protein (FKRP) mutations are the most common cause of dystroglycanopathies known to cause both limb girdle and congenital muscular dystrophy. The P448Lneo- mouse model has a knock-in mutation in the FKRP gene and develops skeletal, respiratory, and cardiac muscle disease. METHODS: We studied the natural history of the P448Lneo- mouse model over 9 months and the effects of twice weekly treadmill running. Forelimb and hindlimb grip strength (Columbus Instruments) and overall activity (Omnitech Electronics) assessed skeletal muscle function...
April 6, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29598826/the-complexity-of-titin-splicing-pattern-in-human-adult-skeletal-muscles
#10
Marco Savarese, Per Harald Jonson, Sanna Huovinen, Lars Paulin, Petri Auvinen, Bjarne Udd, Peter Hackman
BACKGROUND: Mutations in the titin gene (TTN) cause a large spectrum of diseases affecting skeletal and/or cardiac muscle. TTN includes 363 coding exons, a repeated region with a high degree of complexity, isoform-specific elements, and metatranscript-only exons thought to be expressed only during fetal development. Although three main classes of isoforms have been described so far, alternative splicing events (ASEs) in different tissues or in different developmental and physiological states have been reported...
March 29, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29549884/role-of-parkin-and-endurance-training-on-mitochondrial-turnover-in-skeletal-muscle
#11
Chris Chin Wah Chen, Avigail T Erlich, David A Hood
BACKGROUND: Parkin is a ubiquitin ligase that is involved in the selective removal of dysfunctional mitochondria. This process is termed mitophagy and can assist in mitochondrial quality control. Endurance training can produce adaptations in skeletal muscle toward a more oxidative phenotype, an outcome of enhanced mitochondrial biogenesis. It remains unknown whether Parkin-mediated mitophagy is involved in training-induced increases in mitochondrial content and function. Our purpose was to determine a role for Parkin in maintaining mitochondrial turnover in muscle, and its requirement in mediating mitochondrial biogenesis following endurance exercise training...
March 17, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29514713/a-need-for-nad-in-muscle-development-homeostasis-and-aging
#12
REVIEW
Michelle F Goody, Clarissa A Henry
Skeletal muscle enables posture, breathing, and locomotion. Skeletal muscle also impacts systemic processes such as metabolism, thermoregulation, and immunity. Skeletal muscle is energetically expensive and is a major consumer of glucose and fatty acids. Metabolism of fatty acids and glucose requires NAD+ function as a hydrogen/electron transfer molecule. Therefore, NAD+ plays a vital role in energy production. In addition, NAD+ also functions as a cosubstrate for post-translational modifications such as deacetylation and ADP-ribosylation...
March 7, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29510741/a-missense-mutation-in-myh1-is-associated-with-susceptibility-to-immune-mediated-myositis-in-quarter-horses
#13
Carrie J Finno, Giuliana Gianino, Sudeep Perumbakkam, Zoë J Williams, Matthew H Bordbari, Keri L Gardner, Erin Burns, Sichong Peng, Sian A Durward-Akhurst, Stephanie J Valberg
BACKGROUND: The cause of immune-mediated myositis (IMM), characterized by recurrent, rapid-onset muscle atrophy in Quarter Horses (QH), is unknown. The histopathologic hallmark of IMM is lymphocytic infiltration of myofibers. The purpose of this study was to identify putative functional variants associated with equine IMM. METHODS: A genome-wide association (GWA) study was performed on 36 IMM QHs and 54 breed matched unaffected QHs from the same environment using the Equine SNP50 and SNP70 genotyping arrays...
March 6, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29510724/diversification-of-the-muscle-proteome-through-alternative-splicing
#14
REVIEW
Kiran Nakka, Claudia Ghigna, Davide Gabellini, F Jeffrey Dilworth
BACKGROUND: Skeletal muscles express a highly specialized proteome that allows the metabolism of energy sources to mediate myofiber contraction. This muscle-specific proteome is partially derived through the muscle-specific transcription of a subset of genes. Surprisingly, RNA sequencing technologies have also revealed a significant role for muscle-specific alternative splicing in generating protein isoforms that give specialized function to the muscle proteome. MAIN BODY: In this review, we discuss the current knowledge with respect to the mechanisms that allow pre-mRNA transcripts to undergo muscle-specific alternative splicing while identifying some of the key trans-acting splicing factors essential to the process...
March 6, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29477142/hdac4-preserves-skeletal-muscle-structure-following-long-term-denervation-by-mediating-distinct-cellular-responses
#15
Eva Pigna, Alessandra Renzini, Emanuela Greco, Elena Simonazzi, Stefania Fulle, Rosa Mancinelli, Viviana Moresi, Sergio Adamo
BACKGROUND: Denervation triggers numerous molecular responses in skeletal muscle, including the activation of catabolic pathways and oxidative stress, leading to progressive muscle atrophy. Histone deacetylase 4 (HDAC4) mediates skeletal muscle response to denervation, suggesting the use of HDAC inhibitors as a therapeutic approach to neurogenic muscle atrophy. However, the effects of HDAC4 inhibition in skeletal muscle in response to long-term denervation have not been described yet...
February 24, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29463296/nilotinib-impairs-skeletal-myogenesis-by-increasing-myoblast-proliferation
#16
Osvaldo Contreras, Maximiliano Villarreal, Enrique Brandan
BACKGROUND: Tyrosine kinase inhibitors (TKIs) are effective therapies with demonstrated antineoplastic activity. Nilotinib is a second-generation FDA-approved TKI designed to overcome Imatinib resistance and intolerance in patients with chronic myelogenous leukemia (CML). Interestingly, TKIs have also been shown to be an efficient treatment for several non-malignant disorders such fibrotic diseases, including those affecting skeletal muscles. METHODS: We investigated the role of Nilotinib on skeletal myogenesis using the well-established C2C12 myoblast cell line...
February 20, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29444710/a-novel-in-vitro-model-for-the-assessment-of-postnatal-myonuclear-accretion
#17
Anita Kneppers, Lex Verdijk, Chiel de Theije, Mark Corten, Ellis Gielen, Luc van Loon, Annemie Schols, Ramon Langen
BACKGROUND: Due to the post-mitotic nature of myonuclei, postnatal myogenesis is essential for skeletal muscle growth, repair, and regeneration. This process is facilitated by satellite cells through proliferation, differentiation, and subsequent fusion with a pre-existing muscle fiber (i.e., myonuclear accretion). Current knowledge of myogenesis is primarily based on the in vitro formation of syncytia from myoblasts, which represents aspects of developmental myogenesis, but may incompletely portray postnatal myogenesis...
February 14, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29386054/myoblast-fusion-confusion-the-resolution-begins
#18
REVIEW
Srihari C Sampath, Srinath C Sampath, Douglas P Millay
The fusion of muscle precursor cells is a required event for proper skeletal muscle development and regeneration. Numerous proteins have been implicated to function in myoblast fusion; however, the majority are expressed in diverse tissues and regulate numerous cellular processes. How myoblast fusion is triggered and coordinated in a muscle-specific manner has remained a mystery for decades. Through the discovery of two muscle-specific fusion proteins, Myomaker and Myomerger-Minion, we are now primed to make significant advances in our knowledge of myoblast fusion...
January 31, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29329560/overexpression-of-the-double-homeodomain-protein-dux4c-interferes-with-myofibrillogenesis-and-induces-clustering-of-myonuclei
#19
Céline Vanderplanck, Alexandra Tassin, Eugénie Ansseau, Sébastien Charron, Armelle Wauters, Céline Lancelot, Kelly Vancutsem, Dalila Laoudj-Chenivesse, Alexandra Belayew, Frédérique Coppée
BACKGROUND: Facioscapulohumeral muscular dystrophy (FSHD) is associated with DNA hypomethylation at the 4q35 D4Z4 repeat array. Both the causal gene DUX4 and its homolog DUX4c are induced. DUX4c is immunodetected in every myonucleus of proliferative cells, while DUX4 is present in only 1/1000 of myonuclei where it initiates a gene deregulation cascade. FSHD primary myoblasts differentiate into either atrophic or disorganized myotubes. DUX4 expression induces atrophic myotubes and associated FSHD markers...
January 12, 2018: Skeletal Muscle
https://www.readbyqxmd.com/read/29304851/development-of-the-excitation-contraction-coupling-machinery-and-its-relation-to-myofibrillogenesis-in-human-ipsc-derived-skeletal-myocytes
#20
Jeanne Lainé, Gunnar Skoglund, Emmanuel Fournier, Nacira Tabti
BACKGROUND: Human induced pluripotent stem cells-derived myogenic progenitors develop functional and ultrastructural features typical of skeletal muscle when differentiated in culture. Besides disease-modeling, such a system can be used to clarify basic aspects of human skeletal muscle development. In the present study, we focus on the development of the excitation-contraction (E-C) coupling, a process that is essential both in muscle physiology and as a tool to differentiate between the skeletal and cardiac muscle...
January 5, 2018: Skeletal Muscle
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