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Related Concept Videos

Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

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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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Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

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Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
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Cross-bridge Cycle01:26

Cross-bridge Cycle

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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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Myasthenia Gravis: Overview and Treatment01:20

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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.
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Classification of Skeletal Muscle Relaxants01:28

Classification of Skeletal Muscle Relaxants

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Skeletal muscle relaxants are a group of drugs that can reduce muscle stiffness and induce temporary paralysis to relieve pain. These agents can act centrally to reduce muscle tone or spasms in painful conditions such as multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS), or spinal injuries; they are called antispasmodics or spasmolytics.
Peripherally acting skeletal muscle relaxants interfere with the neurotransmission at the neuromuscular end plate to induce paralysis during...
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Chemical Synapses01:26

Chemical Synapses

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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.
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...
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Related Experiment Video

Updated: Apr 11, 2026

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

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Spinal muscular atrophies.

Basil T Darras1

  • 1Division of Clinical Neurology, Department of Neurology, Boston Children's Hospital, 300 Longwood Avenue, Fegan 11, Boston, MA 02115, USA.

Pediatric Clinics of North America
|May 30, 2015
PubMed
Summary

Spinal muscular atrophies (SMAs) are genetic motor neuron diseases causing muscle weakness. Research is active, with ongoing clinical trials offering hope for treatments and improved care for SMA patients.

Keywords:
5q SMADubowitz diseaseKugelberg-Welander diseaseNon-5q SMAsSpinal muscular atrophySurvival of motor neuron proteinWerdnig-Hoffmann disease

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A Functional Motor Unit in the Culture Dish: Co-culture of Spinal Cord Explants and Muscle Cells
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Last Updated: Apr 11, 2026

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Area of Science:

  • Neurology
  • Genetics
  • Molecular Biology

Background:

  • Spinal muscular atrophies (SMAs) are hereditary degenerative disorders affecting lower motor neurons.
  • Proximal 5q SMA, the most common genetic cause of infant mortality, results from reduced survival motor neuron (SMN) protein levels.
  • This condition is inherited in an autosomal recessive pattern due to mutations in the SMN1 gene.

Purpose of the Study:

  • To review the current understanding of Spinal Muscular Atrophy (SMA).
  • To highlight the unique genetic structure of the SMN gene as a therapeutic target.
  • To discuss the active translational research and ongoing clinical trials for SMA.

Main Methods:

  • Review of existing literature on SMA genetics and molecular pathology.
  • Analysis of the unique structural features of the SMN gene.
  • Summary of ongoing translational research efforts and clinical trial progress.

Main Results:

  • SMA is caused by decreased SMN protein, linked to SMN1 gene mutations.
  • The SMN gene's unique structure offers potential therapeutic intervention points.
  • Active research and trials indicate progress in SMA treatment development.

Conclusions:

  • While no definitive cure exists, the field of SMA research is dynamic.
  • Ongoing clinical trials and advances in supportive care provide hope for better outcomes.
  • Targeting the SMN gene presents a promising therapeutic strategy for SMA.