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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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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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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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Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

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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.
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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Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

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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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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: Dec 7, 2025

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

Jessica Rose Nance

    Continuum (Minneapolis, Minn.)
    |October 1, 2020
    PubMed
    Summary

    Spinal muscular atrophy (SMA) treatments have advanced significantly, with gene therapies improving outcomes, especially when initiated early. Continued supportive care remains crucial for managing ongoing symptoms in SMA patients.

    Area of Science:

    • Neurology
    • Genetics
    • Molecular Biology

    Background:

    • Spinal muscular atrophy (SMA) is a rare genetic neuromuscular disorder characterized by the loss of motor neurons, leading to progressive muscle weakness and atrophy.
    • The underlying cause of SMA is a deficiency in the survival motor neuron (SMN) protein, primarily due to mutations in the SMN1 gene.

    Observation:

    • Recent breakthroughs in understanding SMA pathophysiology and genetics have led to the development of novel therapeutic strategies.
    • Three gene-targeted therapies are now FDA-approved: onasemnogene abeparvovec-xioi for SMN1 gene replacement, and nusinersen and risdiplam to enhance SMN2 gene expression.

    Findings:

    • These innovative treatments have demonstrated significant efficacy, particularly when administered early in life, with the most pronounced benefits observed in neonates.

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  • While motor function has improved, patients, especially older individuals, still experience considerable weakness and necessitate ongoing monitoring for respiratory and orthopedic complications.

    • Implications:

    • The advent of gene-targeted therapies has dramatically altered the clinical trajectory of SMA, offering new hope for affected individuals.
    • Despite therapeutic advancements, comprehensive supportive care remains an indispensable component of managing SMA patients, addressing residual symptoms and improving quality of life.