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Related Concept Videos

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 ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

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The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
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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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Indirect-Acting Cholinergic Agonists: Pharmacological Actions01:30

Indirect-Acting Cholinergic Agonists: Pharmacological Actions

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Indirect-acting cholinergic agonists, also known as anticholinesterases, exert their pharmacological effects by enhancing cholinergic transmission in various body parts, including the neuromuscular junction, autonomic cholinergic synapses, and the brain.
At the neuromuscular junction, these agents work by inhibiting the breakdown of acetylcholine, allowing it to remain bound to the receptor and bind to nearby receptors. This process leads to repetitive firing of the endplate, causing muscle...
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Complement System01:27

Complement System

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The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
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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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Antigenic Liposomes for Generation of Disease-specific Antibodies
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Targeting complement system to treat myasthenia gravis.

Ruksana Huda, Erdem Tüzün, Premkumar Christadoss

    Reviews in the Neurosciences
    |April 16, 2014
    PubMed
    Summary

    Complement system activation drives neuromuscular junction destruction in myasthenia gravis. Inhibiting the complement cascade, particularly the classic pathway, shows promise for treating MG without significant immunosuppression.

    Area of Science:

    • Immunology
    • Neuroscience

    Background:

    • Antibody and complement-mediated destruction of the neuromuscular junction (NMJ) is central to myasthenia gravis (MG) and experimental autoimmune MG (EAMG).
    • Pathogenic anti-acetylcholine receptor (AChR) autoantibodies bind complement factors, forming membrane attack complexes (MACs) that damage the NMJ, leading to muscle weakness.

    Purpose of the Study:

    • To investigate the role of the complement system in NMJ destruction in MG and EAMG.
    • To evaluate the therapeutic potential of complement inhibition strategies for MG treatment.

    Main Methods:

    • Studies utilized complement-deficient or complement inhibitor-treated animal models of EAMG.
    • Investigated the effects of inhibiting specific complement components (e.g., C3-C6, C1q, CR1, C6, C5) and naturally occurring inhibitors (DAF).

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  • Assessed EAMG induction, susceptibility, cytokine production, and immunoglobulin isotypes.

    • Main Results:

    • Complement-deficient or inhibitor-treated animals showed high resistance to EAMG induction.
    • Deficiency of decay-accelerating factor (DAF) increased EAMG susceptibility.
    • Complement inhibition resulted in minimal immunosuppression, with only marginal reductions in certain cytokines and immunoglobulin isotypes.
    • A preliminary clinical trial of C5 inhibitor eculizumab showed potential for MG treatment.

    Conclusions:

    • Inhibition of the classic complement pathway (CCP) alone is sufficient to suppress EAMG.
    • Targeting the CCP offers a potential therapeutic strategy for MG, avoiding broad immunosuppressive side effects.
    • Non-antibody-based approaches, like siRNA targeting C2, are effective in EAMG treatment.
    • MG complement inhibition strategies may benefit other complement-mediated autoimmune diseases.