Name: ALS - Motor Neuron Disease: Mechanism and Development of New Therapies (Video) | JoVE Journal
Uploaded: 2007-07-29T00:00:00.000+00:00
Duration: 15 min 48 s
Description: Explore ALS pathology and motor neuron degeneration mechanisms with Jeffrey Rothstein. Discover advances in therapeutic strategies for this progressive neurodegenerative disease.

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als - motor neuron disease: mechanism and development of new therapies

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

Jeffrey D. Rothstein speaks about the pathology and mechanisms underlying amyotrophic lateral sclerosis or ALS, advances in ALS research, and current strategies towards the development of therapies.

My name's Jeff Rothstein. I'm a professor of neurology and neuroscience at Johns Hopkins University. So I'm both a neurologist and a neuroscientist.And on the neurology side of my life I study and work clinically on a LS, which is a neuromuscular disease, atrophic lateral sclerosis. On the laboratory side, I try to understand why a LS occurs in terms of the pathophysiology. Why do motor neurons die?Why do cells not work properly, including cells like astrocytes? And then at the very fundamental side, I work on synaptic biology of astrocytes And glutamate transporters. So motor neuron disease is a very general Term for a class of diseases that affect both upper or lower motor neurons.In the United States, we often refer to this as a LS atrophic lateral sclerosis, but a LS is only one of a number of different motor neuron diseases. So as an example, if the disease only affects upper motor neurons, the cells in the cortex, the brain, then it's referred to as primary lateral sclerosis. And when the disease only affects lower motor neurons, those cells only in the spinal cord, it's considered spinal muscular atrophy.These are all much more rare subtypes where the most common disease is

als-motor-neuron-disease-mechanism-and-development-of-new-therapies

Research

JoVE Journal

Biology

In this video we demonstrate a protocol for dissection of the transversus abdominis muscle of the mouse and use immunofluorescence and microscopy to visualize neuromuscular junctions.

Dissection of the Transversus Abdominis Muscle for Whole-mount Neuromuscular Junction Analysis

The efficacy of intramuscular uptake and retrograde transport of molecules to corresponding motor neurons depends on the location of the injection sites with respect to the motor end plates (MEPs). Here, we describe how to locate MEPs on skeletal muscles to optimise retrograde transport of tracers into motor neurons.

Intramuscular Injections Along the Motor End Plates: A Minimally Invasive Approach to Shuttle Tracers Directly into Motor Neurons

We present refined protocols that allow in vivo monitoring of motor unit function in the mouse. Techniques to measure compound muscle action potential (CMAP) and motor unit number estimation (MUNE) in the mouse hind limb muscles innervated by the sciatic nerve are described.

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

Here we describe the delivery of microRNA using a recombinant adeno-associated virus serotype 9 in a mouse model of a neuromuscular disease. A single peripheral administration in mice resulted in sustained miRNA overexpression in muscle and motor neurons, providing an opportunity to study miRNA function and therapeutic potential in vivo.

Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 to Treat Neuromuscular Diseases in Rodents

The measurement of nerve conduction is a useful tool to assess mouse models of neurodegeneration but it is frequently only applied to stimulate the sciatic nerve in hindlimbs. Here, we describe a technique to measure compound muscle action potential (CMAP) in vivo in the mouse forelimb muscles innervated by the brachial plexus.

In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration

The goal of this technique is to prepare a highly enriched culture of primary motoneurons (MNs) from murine spinal cord. To evaluate the consequences of mutations causing MN diseases, we describe here the isolation of these isolated MNs and their transfection by magnetofection.

Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons

This protocol allows rapid and efficient conversion of induced pluripotent stem cells into motor neurons with a spinal or cranial identity, by ectopic expression of transcription factors from inducible piggyBac vectors.

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

This work presents a protocol to yield homogeneous cell cultures of primary oculomotor, trochlear, and spinal motor neurons. These cultures can be used for comparative analyses of the morphological, cellular, molecular, and electrophysiological characteristics of ocular and spinal motor neurons.

Isolation and Culture of Oculomotor, Trochlear, and Spinal Motor Neurons from Prenatal Islmn:GFP Transgenic Mice

Two related methods are described to visualize subcellular events required for synaptic transmission. These protocols enable the real-time monitoring of the dynamics of presynaptic calcium influx and synaptic vesicle membrane fusion using live-cell imaging of in vitro cultured neurons.

Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis

We describe a method to generate human motor units in commercially available microfluidic devices by co-culturing human induced pluripotent stem cell-derived motor neurons with human primary mesoangioblast-derived myotubes resulting in the formation of functionally active neuromuscular junctions.

Generation of Human Motor Units with Functional Neuromuscular Junctions in Microfluidic Devices

Explore ALS pathology and motor neuron degeneration mechanisms with Jeffrey Rothstein. Discover advances in therapeutic strategies for this progressive neurodegenerative disease.

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

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