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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)

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

 Bioengineering

Cytoplasm

JoVE 10967

The cytoplasm consists of organelles, an aqueous solution called the cytosol, and a framework of protein scaffolds called the cytoskeleton. The cytosol is a rich broth of ions, small organic molecules such as glucose, and macromolecules such as proteins. Several cellular processes including protein synthesis occur in the cytoplasm.

The composition of the cytosol promotes protein folding such that hydrophobic amino acid side chains are oriented away from the aqueous solution and towards the protein core. However, cellular stressors such as aging and changes in pH, temperature, or osmolarity cause protein misfolding. Misfolded proteins may aggregate to form insoluble deposits in the cytoplasm. Insoluble protein aggregates are implicated in neurodegenerative disorders such as Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis. The eukaryotic cytoskeleton consists of three types of filamentous proteins: microtubules, microfilaments, and intermediate filaments. Microtubules–the largest type of filament–are made up of the protein tubulin. Microtubules are dynamic structures that can grow or shrink by adding or removing tubulin molecules from the ends of their strands. They provide structural stability and provide tracks for the transport of proteins and vesicles within the cell. In addition, microtubules play a

 Core: Cell Structure and Function

Cross-bridge Cycle

JoVE 10870

As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.

When ATP, that is attached to the myosin head, is hydrolyzed to ADP, myosin moves into a high energy state bound to actin, creating a cross-bridge. When ADP is released, the myosin head moves to a low energy state, moving actin toward the center of the sarcomere. Binding of a new ATP molecule dissociates myosin from actin. When this ATP is hydrolyzed, the myosin head will bind to actin, this time on a portion of actin closer to the end of the sarcomere. Regulatory proteins troponin and tropomyosin, along with calcium, work together to control the myosin-actin interaction. When troponin binds to calcium, tropomyosin is moved away from the myosin-binding site on actin, allowing myosin and actin to interact and muscle contraction to occur. As a regulator of muscle contraction, calcium concentration is very closely controlled in muscle fibers. Muscle fibers are in close contact with motor neurons. Action potentials in motor neurons cause the release of the neurotransmitter acetylcholine in the vicinity of muscle fibers. This ge

 Core: Musculoskeletal System

Cranial Nerves Exam II (VII-XII)

JoVE 10005

Source:Tracey A. Milligan, MD; Tamara B. Kaplan, MD; Neurology, Brigham and Women's/Massachusetts General Hospital, Boston, Massachusetts, USA


The cranial nerve examination follows the mental status evaluation in a neurological exam. However, the examination begins with observations made upon greeting…

 Physical Examinations III

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

 Immunology and Infection

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

 Medicine

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

 Neuroscience

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

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

 Neuroscience

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

 Developmental Biology

Spontaneous Formation and Rearrangement of Artificial Lipid Nanotube Networks as a Bottom-Up Model for Endoplasmic Reticulum

1Centre for Molecular Medicine Norway, Faculty of Medicine, University of Oslo, 2Department of Chemistry, Faculty of Mathematics and Natural Sciences, University of Oslo, 3Department of Chemistry and Chemical Engineering, Chalmers University of Technology

JoVE 58923

 Bioengineering

Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells

1Department of Integrative Biology and Pharmacology, McGovern Medical School, The University of Texas Health Science Center at Houston, 2Graduate School of Biomedical Sciences, The University of Texas MD Anderson Cancer Center UTHealth, 3Department of Musculoskeletal Oncology, The First Affiliated Hospital of Sun Yat-sen University, 4Women's Health Institute, Cleveland Clinic Foundation, 5Center for Stem Cell and Regenerative Medicine, The Brown Foundation Institute of Molecular Medicine for the Prevention of Human Diseases, The University of Texas Health Science Center at Houston, 6Center for Precision Health, School of Biomedical Informatics and School of Public Health, The University of Texas Health Science Center at Houston

JoVE 57664

 Cancer Research

Targeted Next-generation Sequencing and Bioinformatics Pipeline to Evaluate Genetic Determinants of Constitutional Disease

1Robarts Research Institute, Schulich School of Medicine and Dentistry, Western University, 2Department of Biochemistry, Schulich School of Medicine and Dentistry, Western University, 3Analytic and Translational Genetics Unit, Center for Genomic Medicine, Harvard Medical School, Massachusetts General Hospital, Stanley Centre for Psychiatric Research, Broad Institute of MIT and Harvard, 4Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, 5School of Medicine, Faculty of Health Sciences, Queen's University, 6Faculty of Medicine, Department of Biochemistry, Microbiology and Immunology, University of Ottawa, 7CHEO Research Institute, Faculty of Medicine, University of Ottawa, 8Department of Clinical Neurological Sciences, Western University, 9Division of Neurology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, 10Division of Neurology, Department of Medicine, University of Toronto, 11Morton and Gloria Shulman Movement Disorders Centre, Toronto Western Hospital, 12Department of Clinical Neurological Sciences, Schulich School of Medicine and Dentistry, Western University, 13Parkwood Institute, St. Joseph's Health Care, 14Department of Medicine, Division of Neurology, McMaster University, 15Division of Neurology, Department of Medicine, Baycrest Health Sciences, 16Canadian Partnership for Stroke Recovery Sunnybrook Site, Sunnybrook Health Science Centre, University of Toronto

JoVE 57266

 Genetics

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

 Bioengineering

Tracking Superparamagnetic Iron Oxide-Labeled Mesenchymal Stem Cells using MRI after Intranasal Delivery in a Traumatic Brain Injury Murine Model

1Ph.D. Program for Neural Regenerative Medicine, College of Medical Science and Technology, Taipei Medical University and National Health Research Institutes, 2Center for Neurotrauma and Neuroregeneration, Taipei Medical University, 3TMU Neuroscience Research Center, Taipei Medical University, 4Department of Neurosurgery, Taipei Medical University Hospital, 5Department of Surgery, School of Medicine, College of Medicine, Taipei Medical University

Video Coming Soon

JoVE 60450

 JoVE In-Press

Low-Cost Gait Analysis for Behavioral Phenotyping of Mouse Models of Neuromuscular Disease

1Department of Neurology, Duke University School of Medicine, 2Duke Center for Neurodegeneration & Neurotherapeutics, Duke University School of Medicine, 3Biomedical Sciences Graduate Program, University of California San Diego, 4Department of Neurobiology, Duke University School of Medicine, 5Department of Cell Biology, Duke University School of Medicine

JoVE 59878

 Behavior

Conversion of Human Induced Pluripotent Stem Cells (iPSCs) into Functional Spinal and Cranial Motor Neurons Using PiggyBac Vectors

1Department of Biology and Biotechnology Charles Darwin, Sapienza University of Rome, Italy, 2Center for Life Nano Science, Istituto Italiano di Tecnologia, Italy, 3Laboratory of Stem Cell biology and Molecular Embryology, The Rockefeller University, USA, 4Department of Physiology and Pharmacology, Sapienza University of Rome, Italy

JoVE 59321

 Neuroscience

Characterizing Histone Post-translational Modification Alterations in Yeast Neurodegenerative Proteinopathy Models

1Chemistry Department, Brooklyn College, 2Ph.D. Program in Biochemistry, Graduate Center of the City University of New York, 3Ph.D. Program in Chemistry, Graduate Center of the City University of New York, 4Ph.D. Programs in Chemistry, Biochemistry, and Biology, Graduate Center of the City University of New York

JoVE 59104

 Genetics
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