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  1. Methodology

    Characterization of a Dmd EGFP reporter mouse as a tool to investigate dystrophin expression

    Dystrophin is a rod-shaped cytoplasmic protein that provides sarcolemmal stability as a structural link between the cytoskeleton and the extracellular matrix via the dystrophin-associated protein complex (DAPC...

    Mina V. Petkova, Susanne Morales-Gonzales, Karima Relizani, Esther Gill, Franziska Seifert, Josefine Radke, Werner Stenzel, Luis Garcia, Helge Amthor and Markus Schuelke

    Skeletal Muscle 2016 6:25

    Published on: 5 July 2016

  2. Research

    Progressive impairment of CaV1.1 function in the skeletal muscle of mice expressing a mutant type 1 Cu/Zn superoxide dismutase (G93A) linked to amyotrophic lateral sclerosis

    Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder that is typically fatal within 3–5 years of diagnosis. While motoneuron death is the defining characteristic of ALS, the events ...

    Donald Beqollari, Christin F. Romberg, Gabriella Dobrowolny, Martina Martini, Andrew A. Voss, Antonio Musarò and Roger A. Bannister

    Skeletal Muscle 2016 6:24

    Published on: 23 June 2016

  3. Research

    Treatment with rGDF11 does not improve the dystrophic muscle pathology of mdx mice

    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 ...

    Fabrizio Rinaldi, Yu Zhang, Ricardo Mondragon-Gonzalez, Jeffrey Harvey and Rita C. R. Perlingeiro

    Skeletal Muscle 2016 6:21

    Published on: 14 June 2016

  4. Research

    Four-week rapamycin treatment improves muscular dystrophy in a fukutin-deficient mouse model of dystroglycanopathy

    Secondary dystroglycanopathies are a subset of muscular dystrophy caused by abnormal glycosylation of α-dystroglycan (αDG). Loss of αDG functional glycosylation prevents it from binding to laminin and other ex...

    Steven J. Foltz, Junna Luan, Jarrod A. Call, Ankit Patel, Kristen B. Peissig, Marisa J. Fortunato and Aaron M. Beedle

    Skeletal Muscle 2016 6:20

    Published on: 2 June 2016

  5. Research

    Characterization of a multiprotein complex involved in excitation-transcription coupling of skeletal muscle

    Electrical activity regulates the expression of skeletal muscle genes by a process known as “excitation-transcription” (E-T) coupling. We have demonstrated that release of adenosine 5′-triphosphate (ATP) durin...

    Manuel Arias-Calderón, Gonzalo Almarza, Alexis Díaz-Vegas, Ariel Contreras-Ferrat, Denisse Valladares, Mariana Casas, Héctor Toledo, Enrique Jaimovich and Sonja Buvinic

    Skeletal Muscle 2016 6:15

    Published on: 11 April 2016

  6. Research

    The SH3 and cysteine-rich domain 3 (Stac3) gene is important to growth, fiber composition, and calcium release from the sarcoplasmic reticulum in postnatal skeletal muscle

    The SH3 and cysteine-rich domain 3 (Stac3) gene is specifically expressed in the skeletal muscle. Stac3 knockout mice die perinatally. In this study, we determined the potential role of Stac3 in postnatal skeleta...

    Xiaofei Cong, Jonathan Doering, Davi A. G. Mazala, Eva R. Chin, Robert W. Grange and Honglin Jiang

    Skeletal Muscle 2016 6:17

    Published on: 11 April 2016

  7. Review

    The beneficial role of proteolysis in skeletal muscle growth and stress adaptation

    Muscle atrophy derived from excessive proteolysis is a hallmark of numerous disease conditions. Accordingly, the negative consequences of skeletal muscle protein breakdown often overshadow the critical nature ...

    Ryan A. V. Bell, Mohammad Al-Khalaf and Lynn A. Megeney

    Skeletal Muscle 2016 6:16

    Published on: 6 April 2016

    The Erratum to this article has been published in Skeletal Muscle 2016 6:19

  8. Research

    Dystrophin-deficient dogs with reduced myostatin have unequal muscle growth and greater joint contractures

    Myostatin (Mstn) is a negative regulator of muscle growth whose inhibition promotes muscle growth and regeneration. Dystrophin-deficient mdx mice in which myostatin is knocked out or inhibited postnatally have a ...

    Joe N. Kornegay, Daniel J. Bogan, Janet R. Bogan, Jennifer L. Dow, Jiahui Wang, Zheng Fan, Naili Liu, Leigh C. Warsing, Robert W. Grange, Mihye Ahn, Cynthia J. Balog-Alvarez, Steven W. Cotten, Monte S. Willis, Candice Brinkmeyer-Langford, Hongtu Zhu, Joe Palandra…

    Skeletal Muscle 2016 6:14

    Published on: 4 April 2016

  9. Research

    Muscle-specific loss of Bmal1 leads to disrupted tissue glucose metabolism and systemic glucose homeostasis

    Diabetes is the seventh leading cause of death in the USA, and disruption of circadian rhythms is gaining recognition as a contributing factor to disease prevalence. This disease is characterized by hyperglyce...

    Brianna D. Harfmann, Elizabeth A. Schroder, Maureen T. Kachman, Brian A. Hodge, Xiping Zhang and Karyn A. Esser

    Skeletal Muscle 2016 6:12

    Published on: 30 March 2016

  10. Research

    Chromatin-wide and transcriptome profiling integration uncovers p38α MAPK as a global regulator of skeletal muscle differentiation

    Extracellular stimuli induce gene expression responses through intracellular signaling mediators. The p38 signaling pathway is a paradigm of the mitogen-activated protein kinase (MAPK) family that, although or...

    Jessica Segalés, Abul B. M. M. K. Islam, Roshan Kumar, Qi-Cai Liu, Pedro Sousa-Victor, F. Jeffrey Dilworth, Esteban Ballestar, Eusebio Perdiguero and Pura Muñoz-Cánoves

    Skeletal Muscle 2016 6:9

    Published on: 15 March 2016

  11. Research

    Prolyl hydroxylase domain 2 deficiency promotes skeletal muscle fiber-type transition via a calcineurin/NFATc1-dependent pathway

    Hypoxia exposure is known to induce an alteration in skeletal muscle fiber-type distribution mediated by hypoxia-inducible factor (HIF)-α. The downstream pathway of HIF-α leading to fiber-type shift, however, ...

    Junchul Shin, Aki Nunomiya, Yasuo Kitajima, Takashi Dan, Toshio Miyata and Ryoichi Nagatomi

    Skeletal Muscle 2016 6:5

    Published on: 5 March 2016

  12. Methodology

    Neuromuscular electrical stimulation promotes development in mice of mature human muscle from immortalized human myoblasts

    Studies of the pathogenic mechanisms underlying human myopathies and muscular dystrophies often require animal models, but models of some human diseases are not yet available. Methods to promote the engraftmen...

    Paraskevi Sakellariou, Andrea O’Neill, Amber L. Mueller, Guido Stadler, Woodring E. Wright, Joseph A. Roche and Robert J. Bloch

    Skeletal Muscle 2016 6:4

    Published on: 27 February 2016

  13. Research

    Attenuated Ca2+ release in a mouse model of limb girdle muscular dystrophy 2A

    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 kno...

    Marino DiFranco, Irina Kramerova, Julio L. Vergara and Melissa Jan Spencer

    Skeletal Muscle 2016 6:11

    Published on: 24 February 2016

  14. Research

    Failed upregulation of TFAM protein and mitochondrial DNA in oxidatively deficient fibers of chronic obstructive pulmonary disease locomotor muscle

    Low mitochondrial content and oxidative capacity are well-established features of locomotor muscle dysfunction, a prevalent and debilitating systemic occurrence in patients with chronic obstructive pulmonary d...

    Yana Konokhova, Sally Spendiff, R. Thomas Jagoe, Sudhakar Aare, Sophia Kapchinsky, Norah J. MacMillan, Paul Rozakis, Martin Picard, Mylène Aubertin-Leheudre, Charlotte H. Pion, Jean Bourbeau, Russell T. Hepple and Tanja Taivassalo

    Skeletal Muscle 2016 6:10

    Published on: 18 February 2016

  15. Methodology

    Alternating bipolar field stimulation identifies muscle fibers with defective excitability but maintained local Ca2+ signals and contraction

    Most cultured enzymatically dissociated adult myofibers exhibit spatially uniform (UNI) contractile responses and Ca2+ transients over the entire myofiber in response to electric field stimuli of either polarity ...

    Erick O. Hernández-Ochoa, Camilo Vanegas, Shama R. Iyer, Richard M. Lovering and Martin F. Schneider

    Skeletal Muscle 2016 6:6

    Published on: 5 February 2016

  16. Research

    Exercise-induced mitochondrial p53 repairs mtDNA mutations in mutator mice

    Human genetic disorders and transgenic mouse models have shown that mitochondrial DNA (mtDNA) mutations and telomere dysfunction instigate the aging process. Epidemiologically, exercise is associated with grea...

    Adeel Safdar, Konstantin Khrapko, James M. Flynn, Ayesha Saleem, Michael De Lisio, Adam P. W. Johnston, Yevgenya Kratysberg, Imtiaz A. Samjoo, Yu Kitaoka, Daniel I. Ogborn, Jonathan P. Little, Sandeep Raha, Gianni Parise, Mahmood Akhtar, Bart P. Hettinga, Glenn C. Rowe…

    Skeletal Muscle 2016 6:7

    Published on: 31 January 2016

  17. Research

    DNA-binding sequence specificity of DUX4

    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 b...

    Yu Zhang, John K. Lee, Erik A. Toso, Joslynn S. Lee, Si Ho Choi, Matthew Slattery, Hideki Aihara and Michael Kyba

    Skeletal Muscle 2016 6:8

    Published on: 28 January 2016

  18. Research

    Decrease of myofiber branching via muscle-specific expression of the olfactory receptor mOR23 in dystrophic muscle leads to protection against mechanical stress

    Abnormal branched myofibers within skeletal muscles are commonly found in diverse animal models of muscular dystrophy as well as in patients. Branched myofibers from dystrophic mice are more susceptible to bre...

    Christophe Pichavant, Thomas J. Burkholder and Grace K. Pavlath

    Skeletal Muscle 2016 6:2

    Published on: 21 January 2016

  19. Review

    The ins and outs of muscle stem cell aging

    Skeletal muscle has a remarkable capacity to regenerate by virtue of its resident stem cells (satellite cells). This capacity declines with aging, although whether this is due to extrinsic changes in the envir...

    Andrew S. Brack and Pura Muñoz-Cánoves

    Skeletal Muscle 2016 6:1

    Published on: 18 January 2016

  20. Research

    Genetic disruption of Ano5 in mice does not recapitulate human ANO5-deficient muscular dystrophy

    Anoctamin 5 (ANO5) is a member of a conserved gene family (TMEM16), which codes for proteins predicted to have eight transmembrane domains and putative Ca2+-activated chloride channel (CaCC) activity. It was rece...

    Jing Xu, Mona El Refaey, Li Xu, Lixia Zhao, Yandi Gao, Kyle Floyd, Tallib Karaze, Paul M. L. Janssen and Renzhi Han

    Skeletal Muscle 2015 5:43

    Published on: 21 December 2015

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