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Related Concept Videos

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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.
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Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
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Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
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Related Experiment Video

Updated: Jun 8, 2025

In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
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Amyotrophic lateral sclerosis represents corticomotoneuronal system failure.

Andrew Eisen1, Steve Vucic2, Matthew C Kiernan3,4

  • 1Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.

Muscle & Nerve
|November 8, 2024
PubMed
Summary

Amyotrophic lateral sclerosis (ALS) uniquely affects humans due to our extensive corticomotoneuronal system. Early symptoms reflect the failure of this system, particularly impacting large neurons like Betz cells.

Keywords:
Betz cellTDP‐43amyotrophic lateral sclerosiscorticofugal excitotoxicitycorticomotoneuron

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Area of Science:

  • Neuroscience
  • Neurology
  • Human Biology

Background:

  • The anterograde corticomotoneuronal hypothesis for amyotrophic lateral sclerosis (ALS) has gained support over decades.
  • Evidence from anatomical, pathological, physiological, neuroimaging, and molecular studies reinforces this hypothesis.
  • The extensive corticomotoneuronal system is a uniquely human trait, aligning with ALS being a human-specific disease.

Purpose of the Study:

  • To review the evidence supporting the corticomotoneuronal hypothesis in ALS.
  • To explain how the corticomotoneuronal system's characteristics contribute to ALS pathogenesis and clinical presentation.
  • To highlight the role of specific neuronal vulnerabilities and molecular mechanisms in ALS.

Main Methods:

  • Review and synthesis of existing anatomical, pathological, physiological, neuroimaging, and molecular biological data.
  • Analysis of the evolutionary and species-specific aspects of the corticomotoneuronal system.
  • Examination of clinical features and "split phenotypes" in relation to corticomotoneuronal projections.

Main Results:

  • The corticomotoneuronal system, particularly prominent in humans, is central to ALS.
  • Early ALS symptoms, including limb dexterity loss and bulbar dysfunction, correlate with the failure of strongly corticomotoneuronally innervated motor units.
  • Large Betz cells and pyramidal neurons are vulnerable to the ALS exposome, with proteasome dysfunction and TDP-43 aggregation exacerbating their failure.

Conclusions:

  • The corticomotoneuronal system is the primary neurological structure underlying ALS manifestations.
  • Understanding system-specific genomes and neural networks is crucial for developing precision medicine for ALS.
  • Targeting the corticomotoneuronal system offers a promising avenue for future ALS therapies.