muscle fibers

Summary

Summary: Large, multinucleate single cells, either cylindrical or prismatic in shape, that form the basic unit of skeletal muscle tissue. They consist of a soft contractile substance enclosed in a tubular sheath. They are derived from the fusion of skeletal myoblasts (MYOBLASTS, SKELETAL) into a syncytium, followed by differentiation.

Top Publications

  1. ncbi The IGF-1/PI3K/Akt pathway prevents expression of muscle atrophy-induced ubiquitin ligases by inhibiting FOXO transcription factors
    Trevor N Stitt
    Regeneron Pharmaceuticals, Inc, 777 Old Saw Mill River Road, Tarrytown, NY 10591, USA
    Mol Cell 14:395-403. 2004
  2. pmc Identification of a novel population of muscle stem cells in mice: potential for muscle regeneration
    Zhuqing Qu-Petersen
    Growth and Development Laboratory, Children s Hospital of Pittsburgh, Department of Orthopaedic Surgery, University of Pittsburgh, PA 15260, USA
    J Cell Biol 157:851-64. 2002
  3. ncbi Isolation of adult mouse myogenic progenitors: functional heterogeneity of cells within and engrafting skeletal muscle
    Richard I Sherwood
    Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA
    Cell 119:543-54. 2004
  4. ncbi IL-4 acts as a myoblast recruitment factor during mammalian muscle growth
    Valerie Horsley
    Graduate Program in Biochemistry, Cell, and Developmental Biology, Emory University, Atlanta, GA 30322, USA
    Cell 113:483-94. 2003
  5. pmc Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with beta-dystroglycan
    Günther A Rezniczek
    Max F Perutz Laboratories, Department of Molecular Cell Biology, University of Vienna, A 1030 Vienna, Austria
    J Cell Biol 176:965-77. 2007
  6. ncbi Expression of tumor necrosis factor-alpha in regenerating muscle fibers in inflammatory and non-inflammatory myopathies
    Satoshi Kuru
    Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai Cho, Showa Ku, Japan
    Acta Neuropathol 105:217-24. 2003
  7. ncbi Cell therapy of alpha-sarcoglycan null dystrophic mice through intra-arterial delivery of mesoangioblasts
    Maurilio Sampaolesi
    Stem Cell Research Institute, H S Raffaele, Via Olgettina 58, 20132 Milan, Italy
    Science 301:487-92. 2003
  8. ncbi Functional in vivo gene transfer into the myofibers of adult skeletal muscle
    Massimo Dona
    Department of Biomedical Sciences, C N R Institute of Neuroscience, Unit for Neuromuscular Biology and Physiopathology, University of Padova, Viale G Colombo 3, I 35121, Padova, Italy
    Biochem Biophys Res Commun 312:1132-8. 2003
  9. ncbi Biological progression from adult bone marrow to mononucleate muscle stem cell to multinucleate muscle fiber in response to injury
    Mark A Labarge
    Baxter Laboratory for Genetic Pharmacology, Department of Microbiology and Immunology, Department of Molecular Pharmacology, Stanford University School of Medicine, CCSR 4215, Stanford, CA 94305, USA
    Cell 111:589-601. 2002
  10. ncbi Loss of dystrophin causes aberrant mechanotransduction in skeletal muscle fibers
    Ashok Kumar
    Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA
    FASEB J 18:102-13. 2004

Detail Information

Publications338 found, 100 shown here

  1. ncbi The IGF-1/PI3K/Akt pathway prevents expression of muscle atrophy-induced ubiquitin ligases by inhibiting FOXO transcription factors
    Trevor N Stitt
    Regeneron Pharmaceuticals, Inc, 777 Old Saw Mill River Road, Tarrytown, NY 10591, USA
    Mol Cell 14:395-403. 2004
    ....
  2. pmc Identification of a novel population of muscle stem cells in mice: potential for muscle regeneration
    Zhuqing Qu-Petersen
    Growth and Development Laboratory, Children s Hospital of Pittsburgh, Department of Orthopaedic Surgery, University of Pittsburgh, PA 15260, USA
    J Cell Biol 157:851-64. 2002
    ..Our results suggest that this novel population of muscle-derived stem cells will significantly improve muscle cell-mediated therapies...
  3. ncbi Isolation of adult mouse myogenic progenitors: functional heterogeneity of cells within and engrafting skeletal muscle
    Richard I Sherwood
    Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA
    Cell 119:543-54. 2004
    ..is thought to occur through the action of myogenic satellite cells located in close association with mature muscle fibers; however, these precursor cells have not been prospectively isolated, and recent studies have suggested that ..
  4. ncbi IL-4 acts as a myoblast recruitment factor during mammalian muscle growth
    Valerie Horsley
    Graduate Program in Biochemistry, Cell, and Developmental Biology, Emory University, Atlanta, GA 30322, USA
    Cell 113:483-94. 2003
    ..These data demonstrate that following myotube formation, myotubes recruit myoblast fusion by secretion of IL-4, leading to muscle growth...
  5. pmc Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with beta-dystroglycan
    Günther A Rezniczek
    Max F Perutz Laboratories, Department of Molecular Cell Biology, University of Vienna, A 1030 Vienna, Austria
    J Cell Biol 176:965-77. 2007
    ..of plectin isoforms during myotube differentiation and their localization to different compartments of muscle fibers, identifying plectins 1 and 1f as sarcolemma-associated isoforms, whereas plectin 1d localizes exclusively to ..
  6. ncbi Expression of tumor necrosis factor-alpha in regenerating muscle fibers in inflammatory and non-inflammatory myopathies
    Satoshi Kuru
    Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai Cho, Showa Ku, Japan
    Acta Neuropathol 105:217-24. 2003
    ..We showed that TNF-alpha mRNA and protein were present in muscle fibers. TNF-alpha-positive fibers were observed in all cases of PM, DM and DMD, but were rare or absent in neurogenic ..
  7. ncbi Cell therapy of alpha-sarcoglycan null dystrophic mice through intra-arterial delivery of mesoangioblasts
    Maurilio Sampaolesi
    Stem Cell Research Institute, H S Raffaele, Via Olgettina 58, 20132 Milan, Italy
    Science 301:487-92. 2003
    ..The success of this protocol was mainly due to widespread distribution of donor stem cells through the capillary network, a distinct advantage of this strategy over previous approaches...
  8. ncbi Functional in vivo gene transfer into the myofibers of adult skeletal muscle
    Massimo Dona
    Department of Biomedical Sciences, C N R Institute of Neuroscience, Unit for Neuromuscular Biology and Physiopathology, University of Padova, Viale G Colombo 3, I 35121, Padova, Italy
    Biochem Biophys Res Commun 312:1132-8. 2003
    The postmitotic nature and longevity of skeletal muscle fibers permit stable expression of any transfected gene. Direct in vivo injection of plasmid DNA, in both adult and regenerating muscles, is a safe, inexpensive, and easy approach...
  9. ncbi Biological progression from adult bone marrow to mononucleate muscle stem cell to multinucleate muscle fiber in response to injury
    Mark A Labarge
    Baxter Laboratory for Genetic Pharmacology, Department of Microbiology and Immunology, Department of Molecular Pharmacology, Stanford University School of Medicine, CCSR 4215, Stanford, CA 94305, USA
    Cell 111:589-601. 2002
    ..signals first induce BMDC to occupy the muscle stem cell niche and then to help regenerate mature muscle fibers. The stress-induced progression of BMDC to muscle satellite cell to muscle fiber results in a contribution to ..
  10. ncbi Loss of dystrophin causes aberrant mechanotransduction in skeletal muscle fibers
    Ashok Kumar
    Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA
    FASEB J 18:102-13. 2004
    Dystrophin is a cytoskeletal protein found at the inner surface of skeletal and cardiac muscle fibers. We hypothesize that deficiency of dystrophin increases muscle compliance and causes an aberrant mechanotransduction in muscle fibers...
  11. ncbi Motor unit recruitment strategies investigated by surface EMG variables
    Dario Farina
    Centro di Bioingegneria, Department of Electronics, Politecnico di Torino, Torino 10129, Italy
    J Appl Physiol (1985) 92:235-47. 2002
    ..units (MUs) are recruited by the central nervous system in an orderly manner starting with the smallest, with muscle fibers that usually show the lowest conduction velocity (CV)...
  12. pmc Regulation of muscle fiber type and running endurance by PPARdelta
    Yong Xu Wang
    Gene Expression Laboratory, Salk Institute, La Jolla, California, USA
    PLoS Biol 2:e294. 2004
    ..receptor delta (PPARdelta) in skeletal muscle, which induces a switch to form increased numbers of type I muscle fibers. Treatment of wild-type mice with PPARdelta agonist elicits a similar type I fiber gene expression profile in ..
  13. ncbi Homer proteins and InsP(3) receptors co-localise in the longitudinal sarcoplasmic reticulum of skeletal muscle fibres
    M Salanova
    Department of Neuroscience, University of Siena, Siena, Italy
    Cell Calcium 32:193-200. 2002
    ..Thus, in skeletal muscle, Homer proteins may play a role in the organisation of a second Ca(2+) signalling compartment containing the InsP(3)R, but are apparently not involved in the organisation of RyRs at triads...
  14. ncbi Muscle fibre ontogenesis in farm animal species
    Brigitte Picard
    INRA, Unité de Recherche sur les Herbivores, Theix, 63122 Saint Genes Champanelle, France
    Reprod Nutr Dev 42:415-31. 2002
    ..This comparative survey shows that the cellular processes of differentiation are comparable between species, while their timing is usually species specific...
  15. ncbi Xin, an actin binding protein, is expressed within muscle satellite cells and newly regenerated skeletal muscle fibers
    Thomas J Hawke
    School of Kinesiology and Health Science, York Univ, 4700 Keele St, Toronto ON Canada
    Am J Physiol Cell Physiol 293:C1636-44. 2007
    ..Taken together, the present findings support the hypothesis that Xin is expressed within muscle satellite cells during skeletal muscle regeneration and is involved in the regulation of myoblast function...
  16. ncbi Functional improvement of dystrophic muscle by myostatin blockade
    Sasha Bogdanovich
    Department of Physiology and Pennsylvania Muscle Institute, University of Pennsylvania School of Medicine, 3700 Hamilton Walk, Richards A 601, Philadelphia, Pennsylvania 19104 6085, USA
    Nature 420:418-21. 2002
    ....
  17. ncbi A muscleblind knockout model for myotonic dystrophy
    Rahul N Kanadia
    Department of Molecular Genetics and Microbiology, Powell Gene Therapy Center, Gainesville, FL 32610, USA
    Science 302:1978-80. 2003
    ..Our results support the hypothesis that manifestations of DM can result from sequestration of specific RNA binding proteins by a repetitive element expansion in a mutant RNA...
  18. ncbi Effects of aging on muscle fibre type and size
    Michael R Deschenes
    Department of Kinesiology, The College of William and Mary, Center for Excellence in Aging and Geriatric Health, Williamsburg, Virginia 23187 8795, USA
    Sports Med 34:809-24. 2004
    ..Given the demographic trends evident in most western societies, i.e. increased number of those considered aged, management interventions for sarcopenia must become a major goal of the healthcare profession...
  19. ncbi Plectin scaffolds recruit energy-controlling AMP-activated protein kinase (AMPK) in differentiated myofibres
    Martin Gregor
    Department of Molecular Cell Biology, Max F Perutz Laboratories, University of Vienna, A 1030 Vienna, Austria
    J Cell Sci 119:1864-75. 2006
    ..The distinct plectin expression patterns in different fibre types combined with its involvement in the regulation of isoform compositions of AMPK complexes could provide a mechanism whereby cytoarchitecture influences energy homeostasis...
  20. pmc The action potential-evoked sarcoplasmic reticulum calcium release is impaired in mdx mouse muscle fibres
    Christopher E Woods
    Department of Physiology, UCLA School of Medicine, Los Angeles, CA 90095, USA
    J Physiol 557:59-75. 2004
    ..Since the myoplasmic Ca(2+) concentration is a critical regulator of muscle contraction, these results may help to explain the weakness observed in skeletal muscle fibres from mdx mice and, possibly, Duchenne muscular dystrophy patients...
  21. ncbi p66ShcA modulates tissue response to hindlimb ischemia
    Germana Zaccagnini
    Laboratorio di Patologia Vascolare, Istituto Dermopatico dell Immacolata IRCCS, Rome, Italy
    Circulation 109:2917-23. 2004
    ....
  22. ncbi Development of the neuromuscular junction
    Veit Witzemann
    Max Planck Institut fur medizinische Forschung, Jahnstrasse 29, 69120 Heidelberg, Germany
    Cell Tissue Res 326:263-71. 2006
    ..Following the production of muscle fibers, clusters of acetylcholine receptors (AChRs) are concentrated in the central regions of the myofibers via a ..
  23. pmc Skeletal muscle repair by adult human mesenchymal stem cells from synovial membrane
    Cosimo De Bari
    Laboratory for Skeletal Development and Joint Disorders, Dept of Rheumatology, Katholieke Universiteit Leuven, Herestraat 49, 3000 Leuven, Belgium
    J Cell Biol 160:909-18. 2003
    ..When administered into dystrophic muscles of immunosuppressed mdx mice, hSM-MSCs restored sarcolemmal expression of dystrophin, reduced central nucleation, and rescued the expression of mouse mechano growth factor...
  24. pmc Cholesterol and lipid microdomains stabilize the postsynapse at the neuromuscular junction
    Raffaella Willmann
    Department of Neurochemistry, Brain Research Institute, University of Zurich, Zurich, Switzerland
    EMBO J 25:4050-60. 2006
    ....
  25. pmc Stiffness and fraction of Myosin motors responsible for active force in permeabilized muscle fibers from rabbit psoas
    Marco Linari
    Laboratorio di Fisiologia, Dipartimento di Biologia Animale e Genetica, Universita degli Studi di Firenze, Firenze, Italy
    Biophys J 92:2476-90. 2007
    ..The finding that the fraction of myosin heads attached to actin in an isometric contraction is 0.33 rules out the hypothesis of multiple mechanical cycles per ATP hydrolyzed...
  26. ncbi Cryopreservation of mitochondria and mitochondrial function in cardiac and skeletal muscle fibers
    Andrey V Kuznetsov
    Department of Transplant Surgery, University Hospital Innsbruck, Austria
    Anal Biochem 319:296-303. 2003
    ..Mitochondrial function was analyzed after freeze-thawing permeabilized muscle fibers using glycerol and dimethyl sulfoxide as cryoprotectant...
  27. ncbi Clinical course correlates poorly with muscle pathology in nemaline myopathy
    M M Ryan
    Department of Neurology, Genetics Division, Children s Hospital, Boston, MA 02115, USA
    Neurology 60:665-73. 2003
    ..To report pathologic findings in 124 Australian and North American cases of primary nemaline myopathy...
  28. pmc Effects of tetracaine on voltage-activated calcium sparks in frog intact skeletal muscle fibers
    Stephen Hollingworth
    Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, 19104, USA
    J Gen Physiol 127:291-307. 2006
    The properties of Ca(2+) sparks in frog intact skeletal muscle fibers depolarized with 13 mM [K(+)] Ringer's are well described by a computational model with a Ca(2+) source flux of amplitude 2.5 pA (units of current) and duration 4...
  29. ncbi Stretch-induced, steady-state force enhancement in single skeletal muscle fibers exceeds the isometric force at optimum fiber length
    Dilson E Rassier
    Faculty of Kinesiology, Human Performance Laboratory, University of Calgary, 2500, University Dr N W Calgary, AB T2N 1N4, Calgary, Canada
    J Biomech 36:1309-16. 2003
    ....
  30. ncbi Nitric oxide-dependence of satellite stem cell activation and quiescence on normal skeletal muscle fibers
    Ashley C Wozniak
    Department of Human Anatomy and Cell Science, Faculty of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada
    Dev Dyn 236:240-50. 2007
    ..Results have important implications for designing new therapies that target the role of exercise in health, aging, and disease...
  31. ncbi Embryonic myogenesis pathways in muscle regeneration
    Po Zhao
    Center for Genetic Medicine, Children s National Medical Center, Washington, DC, USA
    Dev Dyn 229:380-92. 2004
    ....
  32. ncbi Calcium signaling in isolated skeletal muscle fibers investigated under "Silicone Voltage-Clamp" conditions
    Claude Collet
    Laboratoire de Physiologie des Elements Excitables, Universite Claude Bernard Lyon 1, UMR CNRS 5123, F69622 Villeurbanne, France
    Cell Biochem Biophys 40:225-36. 2004
    ..properties of the plasma membrane and intracellular signals measured with this methodology in mouse skeletal muscle fibers. Focus is given to the potency of this approach to investigate both fundamental aspects of excitation-..
  33. pmc ADAM12 and alpha9beta1 integrin are instrumental in human myogenic cell differentiation
    Peggy Lafuste
    Institut National de la Santé et de la Recherche Médicale EMI 0011, Faculty of Medicine, Paris XII University, Creteil 94010, France
    Mol Biol Cell 16:861-70. 2005
    ..We conclude that both ADAM12 and alpha9beta1 integrin are expressed during postnatal human myogenic differentiation and that their interaction is mainly operative in nascent myotube growth...
  34. ncbi Non-cross-bridge calcium-dependent stiffness in frog muscle fibers
    M A Bagni
    Dipartimento di Scienze Fisiologiche, Universita degli Studi di Firenze, 50134 Florence, Italy
    Am J Physiol Cell Physiol 286:C1353-7. 2004
    ..This stiffness increase may help in maintaining the sarcomere length uniformity under conditions of instability...
  35. ncbi Expansion of revertant fibers in dystrophic mdx muscles reflects activity of muscle precursor cells and serves as an index of muscle regeneration
    Toshifumi Yokota
    Muscle Cell Biology Group, Medical Research Council Clinical Science Centre, Hammersmith Hospital Campus, Imperial College School of Medicine, London University, Du Cane Road, London, W12 0NN, UK
    J Cell Sci 119:2679-87. 2006
    ..This expansion of revertant clusters depicts the cumulative history of regeneration, thus providing a useful index for functional evaluation of therapies that counteract muscle degeneration...
  36. ncbi Loss of myostatin attenuates severity of muscular dystrophy in mdx mice
    Kathryn R Wagner
    Department of Neurology, The Johns Hopkins University, 725 N Wolfe Street, Baltimore, MD 21205, USA
    Ann Neurol 52:832-6. 2002
    ..Mdx mice lacking myostatin were stronger and more muscular than their mdx counterparts. Diaphragm muscle showed less fibrosis and fatty remodeling, suggesting improved muscle regeneration...
  37. ncbi Synchronized reconstitution of muscle fibers, peripheral nerves and blood vessels by murine skeletal muscle-derived CD34(-)/45 (-) cells
    Tetsuro Tamaki
    Muscle Physiology and Cell Biology Unit, Tokai University School of Medicine, Shimokasuya 143, Isehara, Kanagawa, 259 1143, Japan
    Histochem Cell Biol 128:349-60. 2007
    ..model (C57/BL6N wild-type) resulted in the synchronized reconstitution of blood vessels, peripheral nerves and muscle fibers following significant recovery of total muscle mass (57%) and contractile function (55%), whereas the non-cell-..
  38. ncbi Impaired regeneration of dystrophin-deficient muscle fibers is caused by exhaustion of myogenic cells
    M A M Luz
    Departamento de Anatomia, Instituto de Biologia, Universidade Estadual de Campinas, Campinas, SP, Brasil
    Braz J Med Biol Res 35:691-5. 2002
    Duchenne muscular dystrophy is one of the most devastating myopathies. Muscle fibers undergo necrosis and lose their ability to regenerate, and this may be related to increased interstitial fibrosis or the exhaustion of satellite cells...
  39. ncbi Cell lineage tracing during Xenopus tail regeneration
    Cesare Gargioli
    Centre for Regenerative Medicine, Department of Biology and Biochemistry, University of Bath, Bath BA2 7AY, UK
    Development 131:2669-79. 2004
    ..This shows that metaplasia between differentiated cell types does not occur, and that the process of Xenopus tail regeneration is more akin to tissue renewal in mammals than to urodele tail regeneration...
  40. ncbi The adaptive potential of skeletal muscle fibers
    Dirk Pette
    Department of Biology, University of Konstanz, Germany
    Can J Appl Physiol 27:423-48. 2002
    Mammalian skeletal muscle fibers display a great adaptive potential...
  41. ncbi The role of nitric oxide in muscle fibers with oxidative phosphorylation defects
    Celia H Tengan
    Department of Neurology, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Sao Paulo, Brazil
    Biochem Biophys Res Commun 359:771-7. 2007
    ..Our results indicate specific roles of NO in compensatory mechanisms of muscle fibers with mitochondrial deficiency and suggest the participation of nNOS in the signaling process of mitochondrial ..
  42. ncbi Apoptosis in heart and skeletal muscle
    Andy J Primeau
    School of Kinesiology and Health Science, York University, Toronto, Ontario, M3J 1P3
    Can J Appl Physiol 27:349-95. 2002
    ..This review discusses the basic cellular mechanisms of apoptosis, as well as the current evidence of this process in cardiac and skeletal muscle. The need for more work in this area is highlighted, particularly in exercise and training...
  43. ncbi The regulation of Notch signaling controls satellite cell activation and cell fate determination in postnatal myogenesis
    Irina M Conboy
    Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA 94305, USA
    Dev Cell 3:397-409. 2002
    ....
  44. ncbi Deconstructing Pompe disease by analyzing single muscle fibers: to see a world in a grain of sand
    Nina Raben
    Arthritis and Rheumatism Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland 20892 1820, USA
    Autophagy 3:546-52. 2007
    ..of the human disease, we have analyzed vesicles of the lysosomal-degradative pathway in isolated single muscle fibers from Pompe patients...
  45. pmc C-Met expression and mechanical activation of satellite cells on cultured muscle fibers
    Ashley C Wozniak
    Department of Human Anatomy and Cell Science, Faculty of Medicine, University of Manitoba, 730 William Avenue, Winnipeg, Manitoba R3E 0W3, Canada
    J Histochem Cytochem 51:1437-45. 2003
    ..These methods have a strong potential to further dissect the nature of stretch-induced activation and gene expression among characterized populations of individual quiescent and activated satellite cells...
  46. pmc Structural differentiation of skeletal muscle fibers in the absence of innervation in humans
    Simona Boncompagni
    Interuniversitary Institute of Miology, Centro Scienze dell Invecchiamento, Universita degli Studi G D Annunzio, I 66013 Chieti, Italy
    Proc Natl Acad Sci U S A 104:19339-44. 2007
    ..Most importantly, the recovery occurs in the complete absence of motor and sensory innervation and of nerve-derived trophic factors, that is, solely under the influence of muscle activity induced by electrical stimulation...
  47. ncbi Insulin-like growth factor I slows the rate of denervation induced skeletal muscle atrophy
    Thea Shavlakadze
    School of Anatomy and Human Biology, The University of Western Australia, 35, Stirling Highway, Crawley, Perth, WA 6009, Australia
    Neuromuscul Disord 15:139-46. 2005
    ..It is proposed that the protective effect of mIGF-1 on denervated myofibres might be due to reduced protein breakdown...
  48. pmc Increased expression of the ubiquitin-proteasome pathway in murine myotubes by proteolysis-inducing factor (PIF) is associated with activation of the transcription factor NF-kappaB
    A S Whitehouse
    Pharmaceutical Sciences Research Institute, Aston University, Birmingham B4 7ET, UK
    Br J Cancer 89:1116-22. 2003
    ..The results further suggest that EPA may attenuate protein degradation induced by PIF, at least partly, by preventing NF-kappaB accumulation in the nucleus...
  49. pmc Voltage-dependent dynamic FRET signals from the transverse tubules in mammalian skeletal muscle fibers
    Marino DiFranco
    Department of Physiology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA 90095, USA
    J Gen Physiol 130:581-600. 2007
    ..membrane potential changes in the transverse tubular system (TTS) and surface membranes of adult mice skeletal muscle fibers. Farnesylated EGFP or ECFP (EGFP-F and ECFP-F) were used as immobile FRET donors, and either non-fluorescent (..
  50. ncbi Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy
    Ami Mankodi
    Department of Neurology, School of Medicine and Dentistry, University of Rochester, Box 673, 601 Elmwood Avenue, New York 14642, USA
    Mol Cell 10:35-44. 2002
    ..We propose that a transdominant effect of mutant RNA on RNA processing leads to chloride channelopathy and membrane hyperexcitability in DM...
  51. ncbi IGF-1 induces human myotube hypertrophy by increasing cell recruitment
    V Jacquemin
    CNRS UMR 7000 Cytosquelette et Développement, Paris, France
    Exp Cell Res 299:148-58. 2004
    ..In addition, muscle hypertrophy can be triggered in the absence of proliferation by recruiting more mononucleated cells. We propose that IGF-1-induced hypertrophy can involve the recruitment of reserve cells in human skeletal muscle...
  52. ncbi Myoblast migration is regulated by calpain through its involvement in cell attachment and cytoskeletal organization
    Stephane Dedieu
    Laboratoire Biosciences de l Aliment, Universite Bordeaux I, ISTAB USC INRA 429, 33405 Talence Cedex, France
    Exp Cell Res 292:187-200. 2004
    ....
  53. ncbi Mutant ubiquitin UBB+1 is accumulated in sporadic inclusion-body myositis muscle fibers
    P Fratta
    USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA 90017 1912, USA
    Neurology 63:1114-7. 2004
    ..In s-IBM, UBB+1 may be pathogenic by inhibiting proteasome, thereby promoting accumulation of cytotoxic misfolded amyloid-beta and phosphorylated-tau...
  54. pmc Intramembrane charge movement and L-type calcium current in skeletal muscle fibers isolated from control and mdx mice
    C Collet
    Laboratoire de Physiologie des Elements Excitables, Universite Claude Bernard, F69622 Villeurbanne, France
    Biophys J 84:251-65. 2003
    Dystrophin-deficient muscle fibers from mdx mice are believed to suffer from increased calcium entry and elevated submembranous calcium level, the actual source and functional consequences of which remain obscure...
  55. ncbi Inhibition of myostatin in adult mice increases skeletal muscle mass and strength
    Lisa Anne Whittemore
    Musculoskeletal Sciences Department, Wyeth Research, 200 CambridgePark Drive, Cambridge, MA 02140, USA
    Biochem Biophys Res Commun 300:965-71. 2003
    ..These data show for the first time that myostatin acts postnatally as a negative regulator of skeletal muscle growth and suggest that myostatin inhibitors could provide a therapeutic benefit in diseases for which muscle mass is limiting...
  56. pmc Specific interaction of the actin-binding domain of dystrophin with intermediate filaments containing keratin 19
    Michele R Stone
    Department of Physiology, University of Maryland School of Medicine, Baltimore, MD 21201, USA
    Mol Biol Cell 16:4280-93. 2005
    ..Studies in COS-7 cells and in vitro showed that Dys-ABD binds directly and specifically to K19. Expressed in muscle fibers in vivo, K19 accumulated in the myoplasm in structures that contained dystrophin and spectrin and disrupted ..
  57. pmc Interplay of IKK/NF-kappaB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy
    Swarnali Acharyya
    Human Cancer Genetics Program and Department of Molecular Virology, Immunology and Medical Genetics, The Ohio State University College of Medicine, Columbus, Ohio 43210, USA
    J Clin Invest 117:889-901. 2007
    ..IkappaB kinase/NF-kappaB (IKK/NF-kappaB) signaling is persistently elevated in immune cells and regenerative muscle fibers. Ablation of 1 allele of the p65 subunit of NF-kappaB was sufficient to improve pathology in mdx mice, a model ..
  58. pmc Myostatin negatively regulates satellite cell activation and self-renewal
    Seumas McCroskery
    Animal Genomics, AgResearch, Hamilton 2015, New Zealand
    J Cell Biol 162:1135-47. 2003
    ..indicates that there is an increased number of satellite cells per unit length of freshly isolated Mstn-/- muscle fibers. Determination of proliferation rate suggests that this elevation in satellite cell number could be due to ..
  59. pmc The regenerative plasticity of isolated urodele myofibers and its dependence on MSX1
    Anoop Kumar
    Department of Biochemistry and Molecular Biology, University College London, London, United Kingdom
    PLoS Biol 2:E218. 2004
    The conversion of multinucleate postmitotic muscle fibers to dividing mononucleate progeny cells (cellularisation) occurs during limb regeneration in salamanders, but the cellular events and molecular regulation underlying this ..
  60. pmc Depletion "skraps" and dynamic buffering inside the cellular calcium store
    Bradley S Launikonis
    Section of Cellular Signaling, Department of Molecular Biophysics and Physiology, Rush University, 1750 West Harrison Street, Suite 1279JS, Chicago, IL 60612, USA
    Proc Natl Acad Sci U S A 103:2982-7. 2006
    ..When store depletion becomes widespread, the polymers would collapse to increase store [Ca2+] and sustain the concentration gradient that drives release flux...
  61. ncbi Ca2+-independent phospholipase A2 enhances store-operated Ca2+ entry in dystrophic skeletal muscle fibers
    François Xavier Boittin
    Laboratory of Pharmacology, Geneva Lausanne School of Pharmaceutical Sciences, University of Geneva, University of Lausanne, 1211 Geneva 4, Switzerland
    J Cell Sci 119:3733-42. 2006
    ..The Ca2+-independent phospholipase A2 pathway therefore appears as an attractive target to reduce excessive Ca2+ influx and subsequent degeneration occurring in dystrophic fibers...
  62. ncbi Targeted expression of insulin-like growth factor-I reduces early myofiber necrosis in dystrophic mdx mice
    Thea Shavlakadze
    School of Anatomy and Human Biology, University of Western Australia, Crawley, Perth, Western Australia 6009, Australia
    Mol Ther 10:829-43. 2004
    ..This mechanism of action can account for the long-term reduced severity of the dystropathology in mdx mice that overexpress mIGF-1 and provides promising opportunities for therapeutic strategies...
  63. ncbi Relationship between force and stiffness in muscle fibers after stretch
    Dilson E Rassier
    Human Performance Laboratory, Faculty of Kinesiology, University of Calgary, 2500 Univ Dr, Calgary, AB, Canada T2N 1N4
    J Appl Physiol (1985) 99:1769-75. 2005
    ..Because BDM places cross bridges in a weakly bound, pre-powerstroke state, our results further suggest that force enhancement is partially associated with a recruitment of weakly bound cross bridges into a strongly bound state...
  64. ncbi Design and function of superfast muscles: new insights into the physiology of skeletal muscle
    Lawrence C Rome
    Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Annu Rev Physiol 68:193-221. 2006
    ..Hence the locomotory and sound-producing muscles have mutually exclusive designs...
  65. ncbi Regulation of mitochondrial biogenesis in skeletal muscle by CaMK
    Hai Wu
    Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Science 296:349-52. 2002
    ..Thus, a calcium-regulated signaling pathway controls mitochondrial biogenesis in mammalian cells...
  66. ncbi Functional, structural, and chemical changes in myosin associated with hydrogen peroxide treatment of skeletal muscle fibers
    Ewa Prochniewicz
    Department of Biochemistry, University of Minnesota, Jackson Hall 6 155, 321 Church St, Minneapolis, MN 55455, USA
    Am J Physiol Cell Physiol 294:C613-26. 2008
    ..of muscle contractility, we have studied the effects of hydrogen peroxide on permeabilized rabbit psoas muscle fibers, focusing on changes in myosin purified from these fibers...
  67. ncbi Mac-1(low) early myeloid cells in the bone marrow-derived SP fraction migrate into injured skeletal muscle and participate in muscle regeneration
    Koichi Ojima
    Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4 1 1 Ogawa Higashi, Kodaira, Tokyo 187 8502, Japan
    Biochem Biophys Res Commun 321:1050-61. 2004
    ..Taken together, our data suggest that neither HSCs nor mature inflammatory cells, but Mac-1(low) early myeloid cells in the BM-derived SP fraction, play an important role in regenerating skeletal muscles...
  68. ncbi Wnt signaling induces the myogenic specification of resident CD45+ adult stem cells during muscle regeneration
    Anna Polesskaya
    Ottawa Health Research Institute, Molecular Medicine Program, 501 Smyth Road, Ottawa, Ontario, Canada K1H 8L6
    Cell 113:841-52. 2003
    ..Our data therefore suggest that mobilization of resident CD45(+) stem cells is an important factor in regeneration after injury and highlight the Wnt pathway as a potential therapeutic target for degenerative neuromuscular disease...
  69. ncbi When contractile proteins go bad: the sarcomere and skeletal muscle disease
    Nigel G Laing
    Centre for Medical Research, University of Western Australia, West Australian Institute for Medical Research, Nedlands, Western Australia, Australia
    Bioessays 27:809-22. 2005
    ..The affected patients and families hope for help sooner rather than later. The onus is on all scientists researching sarcomeric proteins to help develop treatments...
  70. pmc The pore region of the skeletal muscle ryanodine receptor is a primary locus for excitation-contraction uncoupling in central core disease
    Guillermo Avila
    Department of Biochemistry, Cinvestav I P N, Zacatenco 07360, Mexico City, Mexico
    J Gen Physiol 121:277-86. 2003
    ..These data demonstrate that CCD mutations in exon 102 disrupt release channel permeation to Ca2+ during EC coupling and that this region represents a primary molecular locus for EC uncoupling in CCD...
  71. ncbi Firing rates of motor units during strong dynamic contractions
    Alejandro Del Valle
    The Miami Project to Cure Paralysis, Department of Neurological Surgery, University of Miami Miller School of Medicine, Florida 33136, USA
    Muscle Nerve 32:316-25. 2005
    ..Thus, protective mechanisms may limit motor unit firing rates during forceful lengthening contractions to minimize damage...
  72. ncbi Mechanical stress activates the nuclear factor-kappaB pathway in skeletal muscle fibers: a possible role in Duchenne muscular dystrophy
    Ashok Kumar
    Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA
    FASEB J 17:386-96. 2003
    ..Our results show that mechanical stretch activates the classical NF-kappaB pathway and this pathway could be predominately active in DMD...
  73. ncbi Induction of cachexia in mice by systemically administered myostatin
    Teresa A Zimmers
    Department of Molecular Biology and Genetics, Johns Hopkins School of Medicine, 725 North Wolfe Street, Baltimore, MD 21205, USA
    Science 296:1486-8. 2002
    ..These data indicate that myostatin acts systemically in adult animals and may be a useful pharmacologic target in clinical settings such as cachexia, where muscle growth is desired...
  74. ncbi Intracellular acidosis enhances the excitability of working muscle
    Thomas H Pedersen
    Department of Physiology, University of Aarhus, DK 8000, Denmark
    Science 305:1144-7. 2004
    ..These results implicate chloride ion channels in muscle function and emphasize that intracellular acidosis of muscle has protective effects during muscle fatigue...
  75. ncbi Defective maintenance of intracellular Ca2+ homeostasis is linked to increased muscle fatigability in the MG29 null mice
    Marco A P Brotto
    Department of Physiology and Biophysics, UMDNJ Robert Wood Johnson Medical School, Piscataway, NJ 08854, USA
    Cell Res 14:373-8. 2004
    ..we investigated the properties of Ca2+-uptake and -release in toxin-skinned Extensor Digitorium Longus (EDL) muscle fibers from control and mg29 knockout mice...
  76. ncbi Sarcolemmal ion channels in dystrophin-deficient skeletal muscle fibres
    Bruno Allard
    Physiologie Intégrative, Cellulaire et Moléculaire, UMR CNRS 5123, Université C Bernard Lyon 1, 43 Bd du 11 Novembre 1918, 69622, Villeurbanne Cedex, France
    J Muscle Res Cell Motil 27:367-73. 2006
    ..This paper briefly summarizes the current knowledge of the properties of sarcolemmal ion channels in fully differentiated dystrophin-deficient skeletal muscle fibres...
  77. ncbi Effect of temperature on cross-bridge properties in intact frog muscle fibers
    Barbara Colombini
    Dipartimento di Scienze Fisiologiche, Universita degli Studi di Firenze, Firenze, Italy
    Am J Physiol Cell Physiol 294:C1113-7. 2008
    ..The increased cross-bridge strain accounts for the greater average force developed...
  78. ncbi Exploring the frontiers of therapeutic exon skipping for Duchenne muscular dystrophy by double targeting within one or multiple exons
    Annemieke Aartsma-Rus
    DMD Genetic Therapy Group, Center for Human and Clinical Genetics, Leiden University Medical Center, RC Leiden, The Netherlands
    Mol Ther 14:401-7. 2006
    ..We aimed at inducing larger multiexon-skipping stretches, such as exons 17-51, exons 42-55, and exons 45-59. However, this appeared complicated and may be dependent on cotranscriptional splicing and the size of the flanking introns...
  79. ncbi Reactive nitrogen species induce nuclear factor-kappaB-mediated protein degradation in skeletal muscle cells
    Marina Bar-Shai
    Department of Anatomy and Cell Biology, Rappaport Faculty of Medicine, Technion Israel Institute of Technology, P O Box 9649, Haifa 31096, Israel
    Free Radic Biol Med 40:2112-25. 2006
    ..NF-kappaB inhibition by antioxidants, tyrosine nitration, and molecular inhibitors may be beneficial for decreasing the extent of muscle damage induced by RNS...
  80. pmc Unloaded shortening velocity and myosin heavy chain variations in human laryngeal muscle fibers
    James J Sciote
    Department of Orthodontics, University of Pittsburgh School of Dental Medicine, Pennsylvania 15261 1032, USA
    Ann Otol Rhinol Laryngol 111:120-7. 2002
    ..This study describes the unloaded shortening velocity (V0) of chemically skinned laryngeal muscle fibers measured by the slack test method in relation to MHC content...
  81. ncbi Topology of Homer 1c and Homer 1a in C2C12 myotubes and transgenic skeletal muscle fibers
    Pompeo Volpe
    Dipartimento di Scienze Biomediche Sperimentali dell Università di Padova, Viale G Colombo 3, 35121 Paduva, Italy
    Biochem Biophys Res Commun 316:884-92. 2004
    ..HA1-tagged Homer 1c and HA1-tagged Homer 1a was investigated in C(2)C(12) myotubes and in transgenic skeletal muscle fibers of the adult rat by epifluorescent and confocal microscopy...
  82. ncbi Strength training increases insulin-mediated glucose uptake, GLUT4 content, and insulin signaling in skeletal muscle in patients with type 2 diabetes
    Mads K Holten
    Department of Medical Physiology, The Panum Institute, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark
    Diabetes 53:294-305. 2004
    ..The adaptation is attributable to local contraction-mediated mechanisms involving key proteins in the insulin signaling cascade...
  83. pmc Comparison of simulated and measured calcium sparks in intact skeletal muscle fibers of the frog
    S M Baylor
    Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Gen Physiol 120:349-68. 2002
    Calcium sparks in frog intact skeletal muscle fibers were modeled as stereotypical events that arise from a constant efflux of Ca(2+) from a point source for a fixed period of time (e.g., 2.5 pA of Ca(2+) current for 4...
  84. ncbi Effect of moderate dehydration on torque, electromyography, and mechanomyography
    Tammy K Evetovich
    Laboratory of Applied Physiology, University of Southern Mississippi, School of Human Performance and Recreation, Hattiesburg, Mississippi, USA
    Muscle Nerve 26:225-31. 2002
    ....
  85. ncbi Inhibition of muscle carbonic anhydrase slows the Ca(2+) transient in rat skeletal muscle fibers
    Petra Wetzel
    Zentrum Physiologie, Medizinische Hochschule Hannover, Carl Neuberg Strasse 1, 30625 Hannover, Germany
    Am J Physiol Cell Physiol 283:C1242-53. 2002
    ..We conclude that inhibition of SR-CA by impairing H(+) countertransport is responsible for deceleration of intracellular Ca(2+) transients and contraction times...
  86. pmc Normal myoblast fusion requires myoferlin
    Katherine R Doherty
    Department of Molecular Genetics and Cell Biology, The University of Chicago, Chicago, IL 60637, USA
    Development 132:5565-75. 2005
    ..These data support a role for myoferlin in the maturation of myotubes and the formation of large myotubes that arise from the fusion of myoblasts to multinucleate myotubes...
  87. pmc Different modes of hypertrophy in skeletal muscle fibers
    Angelika C Paul
    Mouse Biology Program, European Molecular Biology Laboratory, 00016 Monterotondo Scalo, Rome, Italy
    J Cell Biol 156:751-60. 2002
    ..In contrast, hypertrophic gracilis anterior muscle, which is multiply innervated, contained longer muscle fibers, with no increase in average diameter, or in centralized nuclei...
  88. ncbi Disodium cromoglycate protects dystrophin-deficient muscle fibers from leakiness
    Maria Julia Marques
    Departamento de Anatomia, Instituto de Biologia, Universidade Estadual de Campinas, Campinas, Sao Paulo 13083 970, Brazil
    Muscle Nerve 37:61-7. 2008
    ..We verified that cromolyn, a mast-cell stabilizer agent, stabilized dystrophic muscle fibers using Evans blue dye as a marker of sarcolemma leakiness...
  89. ncbi Comparison of Fourier and wavelet transform procedures for examining mechanomyographic and electromyographic frequency versus isokinetic torque relationships
    T W Beck
    Department of Nutrition and Health Sciences, Human Performance Laboratory, Center for Youth Fitness and Sports Research, University of Nebraska Lincoln, Lincoln, Nebraska 68588 0229, USA
    Electromyogr Clin Neurophysiol 45:93-103. 2005
    ..67-0.83) and EMG (R2 = 0.72-0.90). The similarities among the CF, MPF, and MDF patterns suggested that Fourier or wavelet transform procedures can be used to examine the patterns of MMG and EMG responses during dynamic muscle actions...
  90. pmc Mitochondrial DNA-deletion mutations accumulate intracellularly to detrimental levels in aged human skeletal muscle fibers
    Entela Bua
    Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison, WI 53706, USA
    Am J Hum Genet 79:469-80. 2006
    ..The number of VL muscle fibers exhibiting mitochondrial electron-transport-system (ETS) abnormalities increased from an estimated 6% at age ..
  91. ncbi Neurotrophic factors enhance the survival of muscle fibers in EDL, but not SOL, after neonatal nerve injury
    Kambiz Mousavi
    Department of Cellular and Molecular Medicine, University of Ottawa, Ontario K1H 8M5, Canada
    Am J Physiol Cell Physiol 283:C950-9. 2002
    ..NT treatments prevented the axotomy-induced loss of muscle mass in both EDL and SOL and of total number of muscle fibers in EDL but not in SOL...
  92. ncbi Reloading of atrophied rat soleus muscle induces tenascin-C expression around damaged muscle fibers
    Martin Fluck
    M E Müller Institute for Biomechanics, Department of Anatomy, University of Bern, 3000 Bern 9, Switzerland
    Am J Physiol Regul Integr Comp Physiol 284:R792-801. 2003
    ..accumulation of tenascin-C and an increase of fibronectin in the endomysium of a proportion (8 and 15%) of muscle fibers. Large increases of tenascin-C (40-fold) and fibronectin (7-fold) mRNA within 1 day of reloading indicates the ..
  93. ncbi Altered mRNA splicing of the skeletal muscle ryanodine receptor and sarcoplasmic/endoplasmic reticulum Ca2+-ATPase in myotonic dystrophy type 1
    Takashi Kimura
    Division of Molecular Bioscience, John Curtin School of Medical Research, Australian National University, PO Box 334, Canberra ACT 2601, Australia
    Hum Mol Genet 14:2189-200. 2005
    ..We suggest that aberrant splicing of RyR1 and SERCA1 mRNAs might contribute to impaired Ca2+ homeostasis in DM1 muscle...
  94. ncbi Differential expression of calcineurin and SR Ca2+ handling proteins in equine muscle fibers during early postnatal growth
    Karin Eizema
    Department of Pathobiology, Division of Anatomy and Physiology, Faculty of Veterinary Medicine, Utrecht University, P O Box 80 158, NL 3508 TD, Utrecht, The Netherlands
    J Histochem Cytochem 55:247-54. 2007
    ..Although equine muscle is already functional at birth, expression patterns of the monitored proteins still show adaptation, depending on the MyHC fiber type...
  95. ncbi Effects of submaximal exercise on adenine nucleotide concentrations in skeletal muscle fibers of horses with polysaccharide storage myopathy
    Erin J Annandale
    Department of Veterinary Population Medicine, College of Veterinary Medicine, University of Minnesota, Saint Paul, MN 55108, USA
    Am J Vet Res 66:839-45. 2005
    ....
  96. ncbi Relationship between utrophin and regenerating muscle fibers in duchenne muscular dystrophy
    Jeong Yun Shim
    Department of Pathology, Yonsei University College of Medicine, 134 Shinchon dong, Seodaemun gu, Seoul 120 752, Korea
    Yonsei Med J 44:15-23. 2003
    ..These results show that utrophin up-regulation is regeneration-associated, and that it is proportional to the quantity of regenerating myofibers, but is not specific for DMD...
  97. ncbi Effect of injecting primary myoblasts versus putative muscle-derived stem cells on mass and force generation in mdx mice
    Gunhild M Mueller
    Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA
    Hum Gene Ther 13:1081-90. 2002
    ..This may, in part, explain the failure of cellular therapy to alter the contractile properties of murine dystrophic muscle...
  98. ncbi Endurance training induces muscle-specific changes in mitochondrial function in skinned muscle fibers
    Yan Burelle
    Department of Zoology, University of British Columbia, Vancouver, British Columbia, Canada V6T 1Z4
    J Appl Physiol (1985) 92:2429-38. 2002
    ....
  99. ncbi In vivo plasmid DNA electroporation resulted in transfection of satellite cells and lasting transgene expression in regenerated muscle fibers
    Baowei Peng
    School of Life Sciences and Biotechnology, Shanghai Jiaotong University, Shanghai, PR China
    Biochem Biophys Res Commun 338:1490-8. 2005
    ..We found that cells with longer gene expression durations were mostly regenerated muscle fibers. In contrast, expression in pre-existing muscle fibers was rather transient...
  100. ncbi Calcium sensitivity of human single muscle fibers following plyometric training
    Laurent Malisoux
    Department of Physical Education and Rehabilitation, Faculty of Medicine, Catholic University of Louvain, Louvain la Neuve, Belgium
    Med Sci Sports Exerc 38:1901-8. 2006
    To study the effect of plyometric training on Ca2+ sensitivity and the influence of troponin T (TnT) isoforms on Ca2+ -activation properties in skinned human muscle fibers.
  101. ncbi Hypertrophic muscle fibers with fissures in power-lifters; fiber splitting or defect regeneration?
    Anders Eriksson
    Department of Integrative Medical Biology, Section for Anatomy, Umea University, 901 87, Umea, Sweden
    Histochem Cell Biol 126:409-17. 2006
    Power-lifters have hypertrophic muscle fibers with fissures seen in cross-sections, called as fiber splitting. Whether this phenomenon is due to real splitting or defective regeneration has not been settled...

Research Grants75

  1. Agrin/alpha 3 Na,K-ATPase signaling at the neuromuscular junction
    Martin A Smith; Fiscal Year: 2012
    ..breathing, and swallowing, depend on the rapid transfer of information between motor neurons and the skeletal muscle fibers they innervate that takes place at a specialized contact called the neuromuscular junction...
  2. Role of troponin T isoforms in nemaline myopathy
    Jian Ping Jin; Fiscal Year: 2012
    ..of neuromuscular disorders characterized by muscle weakness and rod-shaped "nemaline" inclusions in skeletal muscle fibers. All known inherited nemaline myopathies are caused by mutations in sarcomeric thin filament proteins...
  3. MicroRNA Regulation of Macrophage Polarization in Muscle Regeneration
    Paula K Shireman; Fiscal Year: 2013
    ..cells that reside in skeletal muscle and with muscle injury, can proliferate and fuse together or with damaged muscle fibers to regenerate muscle;macrophages are critical to this process...
  4. Activity Dependent Signaling in Adult Skeletal Muscle Fiber Plasticity
    Martin F Schneider; Fiscal Year: 2012
    ..for activity dependent transcriptional regulation of slow fiber type gene expression in adult fast skeletal muscle fibers using a novel culture system in which calcium, transcription factors and other transcriptional modulators can ..
  5. Pbx Homeodomain Proteins in Skeletal Muscle Differentiation
    LISA A MAVES; Fiscal Year: 2011
    PROJECT SUMMARY The differentiation of muscle precursor cells into contractile skeletal muscle fibers is necessary for normal muscle development and regeneration and, when defective, leads to musculoskeletal diseases such as muscular ..
  6. Planning a Multicenter Trial of PDE5A Inhibition for Duchenne Muscular Dystrophy
    Ronald G Victor; Fiscal Year: 2012
    ..and co-workers during the past two decades have shown that loss of sarcolemmal nNOSm renders the diseased muscle fibers susceptible to functional ischemia and implicated skeletal muscle-derived nitric oxide (NO) as a novel ..
  7. Striated Muscle Gene Transcription and Regulatory Gene Cassettes for Gene Therapy
    STEPHEN DENISON HAUSCHKA; Fiscal Year: 2012
    ..it is expressed in skeletal and cardiac muscle, and at varying levels in different muscles &fast/slow muscle fibers. These attributes plus MCK's intrinsically high expression make its components attractive for use in ..
  8. REACTIVE OXYGEN SPECIES: STRESS AND DAMAGE IN OLD MUSCLE
    Susan V Brooks; Fiscal Year: 2012
    ..the Program Project is to understand the mechanisms underlying the age-related deterioration of motor nerves, muscle fibers, and mitochondria responsible for physical frailty...
  9. Targets of a cytidine deaminase required for left-right axis in Xenopus
    Alin Vonica; Fiscal Year: 2010
    ..In addition, APOBEC2 stimulates the differentiation of a multipotent cell line into muscle fibers. Many congenital heart defects are caused by defects in left-right orientation in humans, and degenerative ..
  10. The role of cMET in satellite cells during muscle regeneration
    MICAH TAUBE WEBSTER; Fiscal Year: 2013
    ..and become myoblasts (muscle precursor cells) that accumulate at sites of injury where they fuse to form new muscle fibers. c-Met and Hgf are up-regulated in the muscle during this period, suggesting an involvement of these genes in ..
  11. Excitation-contraction Coupling in Normal and Dystrophic Mammalian Muscle
    Julio L Vergara; Fiscal Year: 2013
    ..This functional characterization will be achieved in muscle fibers from various animal models of human muscular dystrophies...
  12. Generation of Wunen/LPP3-based therapy for muscular dystrophy
    Hannele Ruohola Baker; Fiscal Year: 2010
    ..as mammalian muscle satellite stem cells which is between the basal lamina and the sarcolemma of existing muscle fibers. In toto, we will establish and extend the role of sphingolipid signaling in muscle formation in Drosophila ..
  13. A novel model for studying fetal skeletal muscle development
    Min Du; Fiscal Year: 2009
    ..The proliferation and lineage commitment of pluripotent cells directly affect the number and size of muscle fibers developed...
  14. The Role of Nuclear Receptor PPARbeta in the Regulation of Muscle Fuel Metabolism
    EMILY YVONNE SMITH; Fiscal Year: 2013
    ..increased muscle glucose uptake and utilization, improved exercise endurance, and an increase in slow-twitch muscle fibers. Conversely, PPAR? transgenic mice exhibit a metabolic phenotype similar to that of the obesity-related ..
  15. Crosslink augmentation to resist snoring and obstructive sleep apnea syndrome
    Thomas P Hedman; Fiscal Year: 2013
    ..trauma to the soft palate due to snoring can be expected to increase its passive deformability and damage muscle fibers and peripheral nerve fibers, increasing the tendency for obstruction...
  16. Role of the Giant Protein Titin in Skeletal Muscle Structure and Function
    Henk L Granzier; Fiscal Year: 2013
    ..a focus on atrophic muscle and on Ehlers-Danlos syndrome, a disease in which the matrix that 'glues'muscle fibers to each other is weakened and in which a range of muscle abnormalities is present...
  17. Cellular and Molecular Analysis of Defects at Aging Neuromuscular Synapses
    Joshua R Sanes; Fiscal Year: 2012
    ..Using transgenic mice in which single motor axons and muscle fibers of specific types are selectively labeled we will assess myogenic and neurogenic determinants of sarcopenia...
  18. Myosin ELC, a novel therapeutic target for FHC
    Danuta Szczesna-Cordary; Fiscal Year: 2013
    ..1B) The contractile properties of the mutated myocardium in skinned and intact papillary muscle fibers. (1C) The age-dependent morphology and function of mutated transgenic mouse hearts by histopathology, ..
  19. Notch and Wnt signaling in protection by nandrolone against disuse atrophy
    Christopher Cardozo; Fiscal Year: 2012
    ..producing daughter cells able to commit to the skeletal muscle lineage, form myoblasts, and fuse to form new muscle fibers or contribute to existing ones. Wnt inhibits Notch signaling and stimulates myogenic differentiation...
  20. Respiratory Muscle Weakness in Chronic Inflammation
    Francisco H Andrade; Fiscal Year: 2013
    ..TNF depresses contractile function of diaphragm and stimulates oxidant production by diaphragm muscle fibers. The oxidants appear to cause weakness since interventions that limit oxidant activity prevent loss of force...
  21. Protein Therapeutics for Muscular Dystrophy
    Norio Takizawa; Fiscal Year: 2013
    ..a family of geneti disorders that generally include progressive muscle weakness due to degeneration of the muscle fibers that is linked to either fragility of the membranes that surround muscle cells or a compromised ability to ..
  22. Impact of Exercise on Sarcopenia
    Judd M Aiken; Fiscal Year: 2013
    ..We have shown that age-dependent mtDNA deletion mutations clonally accumulate to high levels in aged skeletal muscle fibers. Concomitant with this increase in aberrant genomes is a loss of complex IV (cytochrome c oxidase;COX-) ..
  23. Structural Dynamics of Cardiac Muscle Contraction
    Brett A Colson; Fiscal Year: 2012
    ..to measure accurately the effects of genetic ablation of cMyBP-C on structural dynamics in skinned cardiac muscle fibers. This approach is based on previous work in the sponsor's laboratory...
  24. Role of ERK1/2 in Neuromuscular Synapses and Myofiber Development in vivo
    Mendell Rimer; Fiscal Year: 2013
    ..However, no gene targeting investigations on the role of ERK1/2 in developing muscle fibers in vivo have been reported to date...
  25. Roles of voltage sensor, S100A1 and calmodulin in skeletal muscle Ca2+ signaling
    Martin F Schneider; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Activation of skeletal muscle fibers, which is a prerequisite for all bodily movement as well as for respiration, is initiated by electrical depolarization of the transverse tubules (TTs), causing ..
  26. MIDBRAIN CIRCUITRY FOR NEURONAL CONTROL OF GAZE
    Paul J May; Fiscal Year: 2012
    ..motor neurons that control the lens and pupil, and the motor neurons that control the multiply innervated muscle fibers (MIFs) in the extraocular muscles. MIFs are highly fatigue resistant and tonically active...
  27. Single Cross-Bridge Kinetics in Transgenic Mouse Hearts Expressing FHC Mutations
    Danuta Szczesna-Cordary; Fiscal Year: 2012
    ..We will examine this hypothesis at the single molecule level in papillary muscle fibers from transgenic mouse hearts which carry disease-causing mutations in the regulatory and/or essential light ..
  28. FINR: A Novel Modulator of Insulin Signaling
    Marc R Montminy; Fiscal Year: 2010
    ..of TRB3 in promoting fatty acid oxidation and blocking glucose uptake in oxidative but not glycolytic skeletal muscle fibers will also be determined using transgenic mice expressing wild-type, AKT- and COP1- interaction defective TRB3 ..
  29. Structure and Function of HAD Phosphatase Partners Dullard and Lipin
    Debra Dunaway-Mariano; Fiscal Year: 2013
    ..are linked to metabolic syndrome and type-2 diabetes as well as acute, recurrent breakdown of skeletal muscle fibers and susceptibility to statin-induced myopathy (suffered by 2 million people in the United States)...
  30. IDENTIFICATION OF GENES ASSOCIATED WITH MYOBLAST FUSION
    Susan M Abmayr; Fiscal Year: 2012
    ..of muscle development requires recognition between different myoblasts and fusion of these cells to form muscle fibers. The focus of our research is to examine the mechanisms and essential genes through which cells find each ..
  31. Role of Target-derived FGFs in Maintaining and Repairing Synapses
    Gregorio Valdez; Fiscal Year: 2013
    ..from the fact that deleterious changes at the NMJ appears to precede death of motor neurons and atrophy of muscle fibers during the progression of normal aging and ALS...
  32. Circulatory system and integrated muscle tissue for drug and tissue toxicity
    George A Truskey; Fiscal Year: 2013
    ..to posts containing a ferrogel that contracts under electromagnetic stimulation and thereby loads the muscle fibers. Endothelium will cover the outside of the three-dimensional muscle cultures, serving as an interface between ..
  33. Androgen targets in a simple behavioral system
    Cynthia L Jordan; Fiscal Year: 2013
    ..Our model however offers the unexpected and novel perspective that AR may act in muscle fibers and not motoneurons to trigger KD, since the AR transgene is expressed only in skeletal muscle fibers and not ..
  34. Synaptic target selection in Drosophila
    KAI G ZINN; Fiscal Year: 2013
    ..that regulate axon guidance in this system have been identified, we know very little about how individual muscle fibers are recognized as targets by motor axons...
  35. Gastroesophageal Antireflux Mechanisms
    MICHAEL RAYMOND RUGGIERI; Fiscal Year: 2013
    ..We compare responses between the following muscle strips: gastric sling muscle fibers, gastric clasp muscle fibers, lower esophageal circular muscle fibers, mid esophageal circular muscle fibers, ..
  36. Tropin T and Excitation-Contraction Coupling in Aging Skeletal Muscle
    Osvaldo Delbono; Fiscal Year: 2013
    ..We have preliminary evidence of a nontraditional role for TnT3, the TnT isoform expressed in fast-twitch muscle fibers. We found full-length (FL)-TnT3 and its fragments in both the nuclear and cytosolic fractions of myofibers ..
  37. The Role of Small Ankyrin 1 in the Sarcoplasmic Reticulum in Skeletal Muscle
    Andrew P Ziman; Fiscal Year: 2011
    ..protein responsible for cytosolic [Ca2+] removal, and disrupts the organization of the network SR in mature muscle fibers without having a significant effect on the organization of the junctional SR...
  38. Structure and function of the fingers tendinous apparatus
    Francisco J Valero-Cuevas; Fiscal Year: 2013
    ..Aim 2: Implement in real time the known connectivity and dynamics of spinal neurons, muscle proprioceptors and muscle fibers of a single afferented muscle. Validate against data in the literature...
  39. Protein Therapeutics for Muscular Dystrophy
    Norio Takizawa; Fiscal Year: 2010
    ..data to show that intra-muscular delivery of recombinant MG53 can ameliorate cardiotoxin-induced damage to the muscle fibers. This project will comprise an effort by TRIM-edicine that will leverage our expertise to pursue the proof-of-..
  40. Mechanisms of Adaptation to Exercise in Health and COPD
    Peter D Wagner; Fiscal Year: 2013
    ..Studies will be performed in both isolated single muscle fibers and intact animals...
  41. Modulation of Muscle Regenerationby Growth Factors
    Elisabeth R Barton; Fiscal Year: 2013
    ..Because muscle fibers are post-mitotic, repair must rely on satellite cells, a stem cell-like population residing close to muscle ..
  42. Molecular mechanisms regulating motility and migration of muscle satellite cells
    DAWN D CORNELISON; Fiscal Year: 2010
    ..a large pool of replacement myoblasts, relocating to the site of the injury, and differentiating to form new muscle fibers. The molecular and cellular processes required for adult myogenesis (regeneration) overlap with, but are ..
  43. Regulation of Muscle Stem Cell Fate
    Helen M Blau; Fiscal Year: 2013
    ..testing their function upon transplantation into muscles revealed an extraordinary potential to contribute to muscle fibers and access and replenish the satellite cell compartment...
  44. Computational Tools to Describe Cardiac Post-MI Structure and Function Remodeling
    Siamak Ardekani; Fiscal Year: 2012
    ..At the micro-structure scale, the normal 3D organization of muscle fibers in the myocardium that spirals around the left ventricle with a angle that is determined by their transmural ..
  45. Molecular Control of Muscle Fuel Metabolism
    DANIEL PATRICK KELLY; Fiscal Year: 2013
    ..absence of training, including increased capacity for muscle glucose oxidation and an increase in slow-twitch muscle fibers. Transcriptional and metabolomic profiling results strongly suggest that whereas PPARa and PPAR2 regulate many ..
  46. Histone deacetylase 4 and neural activity-dependent muscle remodeling and atrophy
    Tso Pang Yao; Fiscal Year: 2012
    ..Interestingly, upon denervation, HDAC4 dissociates from the NMJ and becomes concentrated to the nucleus in muscle fibers. We showed that HDAC4 can repress the expression of contractile, structural and metabolic proteins implicated ..
  47. Molecular basis for skeletal muscle pathophysiology in Pompe's disease
    Jeffrey E Pessin; Fiscal Year: 2013
    ..Glycolytic type II muscle fibers (white) are primarily affected in Pompe's disease whereas oxidative type I muscle fibers (red) are ..
  48. Mechanisms underlying mechanical properties of muscle-tendon units
    Susan V Brooks; Fiscal Year: 2012
    ..to be stiffer and less extensible overall and increasing the likelihood of contraction-induced injury to muscle fibers. Experiments will be performed on tibialis anterior (TA) muscle-tendon units (MTU) and permeabilized single ..
  49. Skeletal muscle biology in rheumatoid arthritis
    Hakan Westerblad; Fiscal Year: 2013
    ..cytokines that stimulate production of nitric oxide (NO) derivatives and reactive oxygen species (ROS) by muscle fibers. The resulting oxidative stress depresses specific force and causes weakness...
  50. FORMATION AND MAINTENANCE OF NEUROMUSCULAR SYNAPSES
    Wesley J Thompson; Fiscal Year: 2012
    ..While most of the area in which the motoneuron terminal contacts muscle fibers is stable in young adult mice, the site where the nerve enters the NMJ is less so...
  51. In-Incubator Live Cell Fluorescence/DIC Time Lapse Imaging System
    Martin F Schneider; Fiscal Year: 2010
    ..research to address key questions relating to events regulating dedifferentiation of cultured adult skeletal muscle fibers, skeletal myotube and myofiber development and formation, surface epithelial cell stratification and ..
  52. Soluble Motogens and Chemoattractants from Damaged Muscle
    DAWN D CORNELISON; Fiscal Year: 2013
    ..quiescent state and proliferate to form a pool of replacement myoblasts, which will differentiate into new muscle fibers or fuse with damaged ones to rapidly and effectively repair the muscle damage...
  53. Molecular Mechanisms of Neuronal-dependent Muscle Plasticity
    GRACIELA ALEXANDRA UNGUEZ; Fiscal Year: 2013
    ..In S. macrurus, some skeletal muscle fibers fully differentiate only to undergo fusion and subsequent extreme modifications in their morphological and ..
  54. ANATOMICAL SPECIALIZATIONS OF THE HUMAN PHARYNX
    Liancai Mu; Fiscal Year: 2012
    ..and neurochemical changes in the sensory and motor nerves and axon terminals innervating the mucosa and muscle fibers in PD pharynx...
  55. Investigating the Molecular Mechanisms of Skeletal Muscle Atrophy
    MICHAEL DYLE; Fiscal Year: 2013
    ..Gadd45a then translocates to nuclei of skeletal muscle fibers and stimulates demethylation of the Cdkn1a gene promoter...
  56. Nox2-Calcium Signaling in Skeletal Muscle
    George G Rodney; Fiscal Year: 2013
    ..redox sensors and a novel tool to measure force and Ca2+ transients simultaneously in living skeletal muscle fibers to address the following specific aims: 1...
  57. Skeletal Muscle Glucose Transport: Exercise and Insulin
    Gregory D Cartee; Fiscal Year: 2013
    ..Aim 2 will use an innovative method to measure GT by single muscle fibers that are matched for fiber type, but differ by expression of wildtype AS160 or AS160 genetically modified to ..
  58. Mechanistic Understanding of Troponin T Role in Cardiac Thin Filament Activation
    Murali Chandra; Fiscal Year: 2012
    ..measure force/ATPase, rate of tension redevelopment and myofiber dynamic stiffness in re- constituted cardiac muscle fibers. Further, complementary studies such as Ca2+ binding kinetics measure- ments and quantitative mathematical ..
  59. Age-Dependent Regulation of Excitation-Contraction Coupling
    Osvaldo Delbono; Fiscal Year: 2013
    ..to decreased DHPR11 expression, contributing to the decline in SR Ca2+ release and specific force in skeletal muscle fibers from senescent mice. Aim 1...
  60. The Dynamics of Synaptic Component at the Neuromuscular Junction of Living Animal
    Mohammed Akaaboune; Fiscal Year: 2013
    ..We are using the neuromuscular junction (NMJ), an excitatory cholinergic synapse between motor neurons and muscle fibers as our model system, because of the significant impact on human health of disorders at this synapse and ..
  61. TROPHIC MANIPULATIONS OF THE OCULOMOTOR SYSTEM
    Christopher S von Bartheld; Fiscal Year: 2010
    ..Experiments were designed to identify trophic factors that either directly affect muscle fibers or provide feedback signals between eye muscles and innervating motor neurons...
  62. Role of RyRs, DHPRs and Ca2+ Entry in the Inclusion Body Myositis
    Alexander Shtifman; Fiscal Year: 2010
    ..He has also demonstrated that chronically elevated levels of 2- amyloid in the muscle fibers from the transgenic mouse models of IBM also exhibit a substantially elevated myoplasmic [Ca2+]i...
  63. The Anatomical Basis of Human Tongue Biomechanics
    Ira Sanders; Fiscal Year: 2001
    ..by staining to show details of muscle structure and insertion into connective tissue; 4) micro dissection of muscle fibers followed by silver and acetylcholinesterase staining to study details of muscle fiber size and shape, motor ..
  64. The Anatomical Basis of Human Tongue Biomechanics
    Ira Sanders; Fiscal Year: 2003
    ..by staining to show details of muscle structure and insertion into connective tissue; 4) micro dissection of muscle fibers followed by silver and acetylcholinesterase staining to study details of muscle fiber size and shape, motor ..
  65. MICROMECHANICS OF RESPIRATORY MUSCLES
    ALADIN BORIEK; Fiscal Year: 1999
    ..hypothesize that the costal diaphragm has an in-series fiber architecture, and tension is transmitted between muscle fibers along their lateral surfaces through the connective tissue...
  66. EMPHYSEMA--INFLUENCES ON DIAPHRAGM FATIGUE RESISTANCE
    Michael Lewis; Fiscal Year: 1992
    ..disease (COPD) resulting in altered diaphragmatic strength and endurance, due to atrophy of fast- twitch muscle fibers corticosteroids, which are often used in COPD, also causes as well as a decrease in the oxidative capacity of ..
  67. MECHANISMS OF MYOGENIC INDUCTION AND PATTERN IN SOMITES
    Frank Stockdale; Fiscal Year: 1999
    ..induce myogenesis in explanted unspecified somites (somites which when cultured alone do not form muscle fibers)...
  68. MYOBLAST GROWTH AND DIFFERENTIATION DURING MUSCLE AGING
    Charlotte Peterson; Fiscal Year: 2000
    ..myoblasts that proliferate and differentiate in response to muscle degeneration thereby regenerating damaged muscle fibers. Muscle regeneration is impaired in aged individuals...
  69. Spectroscopy of myosin light chain domain dynamics
    Osha Roopnarine; Fiscal Year: 2010
    ..Most of these experiments will be performed on skinned muscle fibers, so that structural dynamics and function can be measured simultaneously on the same sample...
  70. Preclinical Trials of NFkappaB Inhibition in the Treatment of Muscular Dystrophy
    C Carlson; Fiscal Year: 2009
    ..nuclear factor-kappaB (NFkB) have distinct beneficial effects in substantially reducing the loss of striated muscle fibers and restoring the resting membrane potential in severely dystrophic (mdx) muscle fibers [2]...
  71. POSTEMBRYONIC DEVELOPMENT OF LEG MOTOR CIRCUITS
    Richard Levine; Fiscal Year: 2000
    ..Motoneurons regulate the differentiation of muscle fibers and retrograde signals from the muscle fibers may be essential for the presynaptic specialization of motor ..
  72. SKELETAL MUSCLE MICROVASCULAR PERFUSION IN HYPERTENSION
    Sarah Gray; Fiscal Year: 1999
    DESCRIPTION: Restoration of muscle function after injury requires the coordinate restoration of muscle fibers and microcirculation, as well as muscle and vascular innervation...
  73. FUNCTIONAL AND MOLECULAR STUDY OF HUMAN MASSETER FIBERS
    James Sciote; Fiscal Year: 2000
    DESCRIPTION (Adapted from the Applicant's Abstract): Mammalian skeletal muscle fibers have been traditionally classified into specific types by characterization of their myofibrillar ATPase and metabolic histochemistry and more recently ..
  74. CELL LINEAGE COMMITMENT IN MAMMALIAN MYOGENESIS
    Jeffrey Miller; Fiscal Year: 1991
    ..Cells first become committed to myogenesis and form muscle fibers in the somites...
  75. LONG MUSCLES WITH SERIES FIBERS--ULTASTRUCTURE
    John Trotter; Fiscal Year: 1991
    ..with parallel architecture such as the hamstrings, sartorius, adductors and abdominal muscles, the individual muscle fibers are shorter than the muscle, and terminate within the muscle by tapering to fine endings...