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Amyotrophic Lateral Sclerosis: A degenerative disorder affecting upper Motor neurons in the brain and lower motor neurons in the brain stem and Spinal cord. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, Fasciculation, hyperreflexia, Dysarthria, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous Astrocytes and atrophy of anterior Spinal nerve roots and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)
 JoVE Bioengineering

Novel Atomic Force Microscopy Based Biopanning for Isolation of Morphology Specific Reagents against TDP-43 Variants in Amyotrophic Lateral Sclerosis

1School for Engineering of Matter, Transport and Energy, Arizona State University, 2Department of Neurology, Georgetown University Medical Center, 3Department of Pathology, Georgetown University Medical Center


JoVE 52584

 JoVE Medicine

A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)

1Department of Speech-Language Pathology, University of Toronto, 2ALS/ MN Clinic, Sunnybrook Health Science Centre, 3Department of Special Education and Communication Disorders, University of Nebraska-Lincoln, 4Department of Neurology, Munroe-Meyer Institute, University of Nebraska Medical Center, 5Department of Neurology, University of Toronto


JoVE 2422

 JoVE Medicine

Adapting Human Videofluoroscopic Swallow Study Methods to Detect and Characterize Dysphagia in Murine Disease Models

1Department of Otolaryngology - Head and Neck Surgery, University of Missouri, 2Department of Communication Science and Disorders, University of Missouri, 3Department of Medicine, University of Missouri


JoVE 52319

 JoVE Immunology and Infection

Antibody Binding Specificity for Kappa (Vκ) Light Chain-containing Human (IgM) Antibodies: Polysialic Acid (PSA) Attached to NCAM as a Case Study

1Department of Neurology, Mayo Clinic, 2Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, 3Center for Regenerative Medicine, Neuroregeneration, Mayo Clinic, 4Division of Neonatal Medicine, Mayo Clinic, 5Department of Pediatric and Adolescent Medicine, Mayo Clinic


JoVE 54139

 JoVE Neuroscience

The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy

1Physiology and Bosch Institute, University of Sydney, 2Motor Neuron Disease Research Group, Australian School of Advanced Medicine, Macquarie University, 3Advanced Microscopy Facility, Bosch Institute, University of Sydney


JoVE 52220

 JoVE Biology

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

 JoVE Behavior

Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles

1Department of Neurology, The Ohio State University Wexner Medical Center, 2Department of Physical Medicine and Rehabilitation, The Ohio State University, 3Department of Neuroscience, The Ohio State University Wexner Medical Center, 4Department of Biochemistry and Pharmacology, The Ohio State University Wexner Medical Center


JoVE 52899

 JoVE Developmental Biology

Imaging Subcellular Structures in the Living Zebrafish Embryo

1Institute of Neuronal Cell Biology, Technische Universität München, 2Cell Biology, Department of Biology, Faculty of Science, Utrecht University, 3Faculty of Biology, Ludwig-Maximilians-Universität-München, 4Adolf-Butenandt-Institute, Biochemistry, Ludwig-Maximilians-Universität-München, 5German Center for Neurodegenerative Diseases, 6Laboratory of Brain Development and Repair, The Rockefeller University


JoVE 53456

 JoVE Neuroscience

Derivation of Glial Restricted Precursors from E13 mice

1Hugo W. Moser Research Institute at Kennedy Krieger, Johns Hopkins University, 2Department of Neurology, Johns Hopkins School of Medicine, 3University of Maryland, 4Experimental Neurology, Biogen Idec, 5The Brain Science Institute, Johns Hopkins School of Medicine, 6Department of Pediatrics, Johns Hopkins School of Medicine


JoVE 3462

 JoVE Bioengineering

Measurement of Maximum Isometric Force Generated by Permeabilized Skeletal Muscle Fibers

1Department of Orthopaedic Surgery, University of Michigan Medical School, 2Department of Molecular & Integrative Physiology, University of Michigan Medical School, 3Department of Biomedical Engineering, University of Michigan Medical School, 4Department of Surgery, Section of Plastic Surgery, University of Michigan Medical School


JoVE 52695

 JoVE Biology

Purification of Transcripts and Metabolites from Drosophila Heads

1Department of Neurology, McKnight Brain Institute, University of Florida, 2Department of Entomology and Nematology, University of Florida, 3Genetics Institute, Department of Molecular Genetics and Microbiology, University of Florida, 4McKnight Brain Institute, Department of Neuroscience, Genetics Institute, Center for Translational Research on Neurodegenerative Diseases, and Center for Movement Disorders and Neurorestoration, University of Florida


JoVE 50245

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