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Related Concept Videos

Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

2.1K
The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
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Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

3.1K
Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
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Parkinson's Disease: Overview01:15

Parkinson's Disease: Overview

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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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Chemical Synapses01:26

Chemical Synapses

12.1K
Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
12.1K
Chemical Synapses01:26

Chemical Synapses

5.0K
Chemical synapses are specialized sites between two neurons or between a neuron and a non-neuronal cell like a muscle, glandular or sensory cell.
Because chemical synapses depend on the release of neurotransmitter molecules from synaptic vesicles to pass on their signal, there is an approximately one millisecond delay between when the axon potential reaches the presynaptic terminal and when the neurotransmitter leads to opening of postsynaptic ion channels. Additionally, this signaling is...
5.0K
Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

2.8K
Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
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Related Experiment Video

Updated: Feb 27, 2026

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

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Motoneuron Disease: Clinical.

Hristelina Ilieva1, Nicholas J Maragakis2

  • 1Department of Neurology, Johns Hopkins University, 855 N. Wolfe St., Rm 248, Baltimore, MD, 21205, USA.

Advances in Neurobiology
|July 5, 2017
PubMed
Summary

Amyotrophic lateral sclerosis (ALS) presents with varied progression and genetic causes. Research is advancing diagnostics and exploring novel therapies like gene and stem cell treatments for this neurodegenerative disease.

Keywords:
DiagnosisExerciseFrontotemporal dementiaGene therapyHeterogeneityNutritionPhenotypeRespiratory managementStem cell therapy

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In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
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Dissection of Single Skeletal Muscle Fibers for Immunofluorescent and Morphometric Analyses of Whole-Mount Neuromuscular Junctions
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Dissection of Single Skeletal Muscle Fibers for Immunofluorescent and Morphometric Analyses of Whole-Mount Neuromuscular Junctions

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Related Experiment Videos

Last Updated: Feb 27, 2026

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

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

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Dissection of Single Skeletal Muscle Fibers for Immunofluorescent and Morphometric Analyses of Whole-Mount Neuromuscular Junctions
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Area of Science:

  • Neuroscience
  • Genetics
  • Biomarker Research

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by neuromuscular weakness.
  • Significant heterogeneity exists in ALS anatomical and temporal progression patterns.
  • Recent genetic discoveries have identified new causative genes for ALS.

Purpose of the Study:

  • To summarize recent advances in understanding ALS heterogeneity and genetics.
  • To highlight the development of novel biomarkers for diagnosis and prognosis.
  • To review emerging therapeutic strategies beyond traditional pharmaceuticals.

Main Methods:

  • Review of recent scientific literature on ALS.
  • Analysis of genetic studies identifying causative genes.
  • Examination of biomarker development initiatives.
  • Survey of ongoing and emerging therapeutic trials.

Main Results:

  • ALS progression is highly variable across individuals.
  • New genes implicated in familial ALS have been identified.
  • Biomarker research is crucial for improved ALS management.
  • Novel therapies including gene therapy and stem cells show promise.

Conclusions:

  • Understanding ALS heterogeneity and genetics is key to targeted treatments.
  • Biomarkers are essential for personalized medicine approaches in ALS.
  • Innovative therapeutic strategies offer new hope for ALS patients.