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Related Concept Videos

Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

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...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

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 leads...
Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

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...
Chemical Synapses01:26

Chemical Synapses

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...
Chemical Synapses01:26

Chemical Synapses

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...
Complement System01:27

Complement System

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 membrane...

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Related Experiment Video

Updated: May 18, 2026

Antigenic Liposomes for Generation of Disease-specific Antibodies
10:31

Antigenic Liposomes for Generation of Disease-specific Antibodies

Published on: October 25, 2018

Cell surface complement regulators moderate experimental myasthenia gravis pathology.

Linda L Kusner1, Jose A Halperin, Henry J Kaminski

  • 1Department of Pharmacology and Physiology, George Washington University, Washington, DC, USA. lkusner@gwu.edu

Muscle & Nerve
|October 9, 2012
PubMed
Summary

Complement regulators CD59 and DAF protect neuromuscular junctions from injury in experimental autoimmune myasthenia gravis. Absence of CD59 or DAF exacerbates disease, highlighting their protective roles.

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The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy
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The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy

Published on: December 26, 2014

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The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy
12:18

The Neuromuscular Junction: Measuring Synapse Size, Fragmentation and Changes in Synaptic Protein Density Using Confocal Fluorescence Microscopy

Published on: December 26, 2014

Area of Science:

  • Immunology
  • Neuroscience
  • Complement System

Background:

  • Intrinsic complement regulators modulate experimental autoimmune myasthenia gravis (EAMG) severity.
  • CD59 is a key complement regulator at the neuromuscular junction (NMJ).
  • The role of CD59b in the absence of CD59a is not fully understood.

Purpose of the Study:

  • To investigate the protective role of CD59 in the absence of CD59a.
  • To evaluate CD59's influence on neuromuscular junction integrity during EAMG.

Main Methods:

  • EAMG was induced using anti-acetylcholine receptor (AChR) monoclonal antibodies.
  • Experiments were conducted in wild-type, CD59ab(-/-), Daf1(-/-), and Daf1(-/-)CD59ab(-/-) mice.
  • Neuromuscular junction injury was assessed via histological analysis of diaphragms.

Main Results:

  • Mice lacking both CD59 and DAF (Daf1(-/-)CD59ab(-/-)) experienced severe weakness and required euthanasia within 24 hours.
  • Histology revealed reduced AChR density, simplified synaptic folds, and mitochondrial disruption in these mice.
  • CD59ab-deficient mice showed mild weakness, while Daf1(-/-) mice exhibited more severe weakness at later time points.
  • NMJs in Daf1(-/-) and CD59ab(-/-) mice showed similar AChR density after EAMG induction.

Conclusions:

  • Both CD59 and DAF protect neuromuscular junctions from complement-mediated injury in passive EAMG.
  • These findings underscore the critical role of complement regulators in maintaining NMJ integrity during autoimmune attack.