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Myotonic Dystrophy: An autosomal dominant neuromuscular disorder which usually presents in early adulthood, characterized by progressive muscular atrophy (most frequently involving the hands, forearms, and face), myotonia, frontal baldness, lenticular opacities, and testicular atrophy. Cardiac conduction abnormalities, diaphragmatic weakness, and mild Intellectual disability may also occur. Congenital myotonic dystrophy is a severe form of this disorder, characterized by neonatal Muscle hypotonia, feeding difficulties, respiratory muscle weakness, and an increased incidence of Intellectual disability. (From Adams et al., Principles of Neurology, 6th ed, pp1423-5; Joynt, Clinical Neurology, 1997, Ch16, pp16-7)

High-throughput Analysis of Locomotor Behavior in the Drosophila Island Assay

1Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, 2Centre for Molecular and Biomolecular Informatics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, 3Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center

JoVE 55892


 Neuroscience

Consensus Brain-derived Protein, Extraction Protocol for the Study of Human and Murine Brain Proteome Using Both 2D-DIGE and Mini 2DE Immunoblotting

1Team Alzheimer & Tauopathies, Jean-Pierre Aubert Research Centre, Inserm UMR 837, 2EA 4308-Department of Reproductive Biology-Spermiology-CECOS, CHRU-Lille, 3EA2686-Laboratorie d'Immunologie, Faculté de Médecine - Pôle Recherche, 4Department of Neurology, CHRU-Lille

JoVE 51339


 Neuroscience

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Quantitative Magnetic Resonance Imaging of Skeletal Muscle Disease

1Institute of Imaging Science, Vanderbilt University, 2Department of Radiology and Radiological Sciences, Vanderbilt University, 3Department of Biomedical Engineering, Vanderbilt University, 4Department of Molecular Physiology and Biophysics, Vanderbilt University, 5Department of Physical Medicine and Rehabilitation, Vanderbilt University, 6Department of Physics and Astronomy, Vanderbilt University

JoVE 52352


 Medicine

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Analysis of Zebrafish Larvae Skeletal Muscle Integrity with Evans Blue Dye

1Program in Genetics & Genome Biology, The Hospital for Sick Children, 2Department of Molecular Genetics, The University of Toronto, 3Program in Genomics of Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, 4Departments of Pediatrics and Neurology, University of Michigan

JoVE 53183


 Developmental Biology

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In Situ Immunofluorescent Staining of Autophagy in Muscle Stem Cells

1Department of Medicine, Institute of Translational Pharmacology, Italian National Research Council, 2Epigenetics and Regenerative Medicine, IRCCS Fondazione Santa Lucia, 3Biological and Environmental Sciences and Engineering Division, King Abdullah University of Science and Technology (KAUST), 4Department of Life Sciences, University of Modena and Reggio Emilia

JoVE 55908


 Biology

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Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies

1Department of Anatomy and Cell Biology, School of Dental Medicine, University of Pennsylvania, 2Department of Physiology, Perelman School of Medicine, University of Pennsylvania, 3Department of Anatomy and Cell Biology, School of Dental Medicine, School of Dental Medicine, University of Pennsylvania

JoVE 50036


 Biology

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Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

1Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, 2Department of Medicine, Vanderbilt University Medical Center, 3Division of Cardiovascular Medicine, Vanderbilt University Medical Center, 4Cardiovascular Institute, Maine Medical Center

JoVE 54005


 Biology

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Assessment of Calcium Sparks in Intact Skeletal Muscle Fibers

1Department of Surgery, Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center, 2Department of Physiology and Cell Biology, Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center, 3Department of Molecular Biophysics and Physiology, Rush University Medical Center, 4Department of Internal Medicine, Davis Heart and Lung Research Institute, The Ohio State University Wexner Medical Center

JoVE 50898


 Biology

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Isolation and Immortalization of Patient-derived Cell Lines from Muscle Biopsy for Disease Modeling

1Department of Cell Biology, UT Southwestern Medical Center, 2National Institute of Neurological Disorders and Stroke, National Institute of Health, 3Division of Pediatric Pathology, Department of Pathology and Laboratory Medicine, Medical College of Wisconsin, 4Division of Genetics and Genomics, Boston Children's Hospital

JoVE 52307


 Medicine

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Tissue Triage and Freezing for Models of Skeletal Muscle Disease

1Division of Pediatric Pathology, Department of Pathology and Laboratory Medicine, Medical College of Wisconsin, 2Department of Physiology and Cell Biology, The Ohio State University, 3Department of Human Nutrition, Foods and Exercise, Virginia Tech, 4Division of Biomedical Informatics, Department of Biostatistics, Department of Computer Science, University of Kentucky, 5Division of Genetics and Genomics, The Manton Center for Orphan Disease Research, Boston Children's Hospital, Harvard Medical School, 6Cure Congenital Muscular Dystrophy, 7Joshua Frase Foundation, 8Department of Rehabilitation Medicine, University of Washington, 9Department of Physiology, University of Arizona

JoVE 51586


 Biology

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A Novel Stretching Platform for Applications in Cell and Tissue Mechanobiology

1Centre for Interdisciplinary NanoPhysics, Department of Physics, University of Ottawa, 2University of Ottawa Heart Institue, University of Ottawa, 3Libin Cardiovascular Institute of Alberta, University of Calgary, 4Department of Biology, University of Ottawa, 5Institute for Science, Society and Policy, University of Ottawa

JoVE 51454


 Bioengineering

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Balance and Coordination Testing

JoVE 5423

Balance and coordination are critical components involved in the control of movement. Many sensory receptors and neural processing units are required to help individuals maintain balance while performing various activities. Deficits in balance and coordination occur in patients suffering from movement disorders or due to aging. Therefore, scientists are trying to understand the pathophysiology behind these conditions. One way to do that is by using rodent models and testing them on behavioral paradigms such as the rotarod or balance beam. This video discusses the currently known neurophysiology behind balance and coordination. Then, we go over protocols to run balance tests in rodents using the rotarod and balance beam. Finally, we'll discuss some current studies utilizing these methods to investigate aging, muscular dystrophy and Parkinson's disease.


 Behavioral Science

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