Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

The Neuromuscular Junction01:19

The Neuromuscular Junction

The nervous system consists of complex motor neuron circuits, including upper motor neurons originating from the cerebral cortex and lower motor neurons starting in the spinal cord, coordinating both voluntary and involuntary movements. Among these, somatic motor neurons activate skeletal muscles and are classified into alpha, beta, and gamma types. Alpha neurons are vital for voluntary movement coordination, while gamma neurons adjust muscle spindle sensitivity, and the function of beta...
Neuromuscular Junction And Blockade01:29

Neuromuscular Junction And Blockade

The site of chemical communication between a motor neuron and a muscle fiber is called the neuromuscular junction (NMJ). The end of the motor neuron at the NMJ divides into a cluster of synaptic end bulbs. The cytoplasm of these bulbs consists of synaptic vesicles enclosing acetylcholine molecules, the principal neurotransmitter released at the NMJ. The region opposite the synaptic bulb that ends in the muscle fiber is called the motor end plate, which has acetylcholine receptors. Within the...
Relaxation of Skeletal Muscles01:29

Relaxation of Skeletal Muscles

The period of muscle contraction primarily influences the duration of stimulation at the neuromuscular junction (NMJ), the presence of free calcium ions in the sarcoplasm, and the availability of energy or ATP to support contractions.
When an action potential reaches the axon terminal, it depolarizes the membrane and opens voltage-gated sodium channels. Sodium ions enter the cell, further depolarizing the presynaptic membrane. This depolarization causes voltage-gated calcium channels to open.
Nondepolarizing (Competitive) Neuromuscular Blockers: Mechanism of Action01:17

Nondepolarizing (Competitive) Neuromuscular Blockers: Mechanism of Action

Nondepolarizing neuromuscular blockers induce paralysis by competitively blocking nicotinic acetylcholine receptors at the muscle end plate. Examples include pancuronium, mivacurium, vecuronium, and rocuronium. These quaternary ammonium derivatives are administered intravenously, are poorly absorbed, and are excreted via the kidneys.
Competitive antagonists prevent acetylcholine from binding to its receptor, inhibiting membrane depolarization. Without conformational changes or intrinsic...
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...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

MuSK cysteine-rich domain antibodies are pathogenic in a mouse model of autoimmune myasthenia gravis.

The Journal of clinical investigation·2025
Same author

Managing surface energy dynamics for enhanced axonal growth: An overview of present and future challenges.

Biophysics reviews·2025
Same author

Stepwise molecular specification of excitatory synapse diversity onto cerebellar Purkinje cells.

Nature neuroscience·2024
Same author

MBNL deficiency in motor neurons disrupts neuromuscular junction maintenance and gait coordination.

Brain : a journal of neurology·2024
Same author

GDF5 as a rejuvenating treatment for age-related neuromuscular failure.

Brain : a journal of neurology·2024
Same author

Molecular Analysis of a Congenital Myasthenic Syndrome Due to a Pathogenic Variant Affecting the C-Terminus of ColQ.

International journal of molecular sciences·2023

Related Experiment Video

Updated: Jun 17, 2026

Physiological Recordings of High and Low Output NMJs on the Crayfish Leg Extensor Muscle
10:00

Physiological Recordings of High and Low Output NMJs on the Crayfish Leg Extensor Muscle

Published on: November 17, 2010

ColQ controls postsynaptic differentiation at the neuromuscular junction.

Séverine M Sigoillot1, Francine Bourgeois, Monique Lambergeon

  • 1Laboratoire de biologie des jonctions neuromusculaires normales et pathologiques, Université Paris Descartes, Inserm U686, 75270 Paris, France.

The Journal of Neuroscience : the Official Journal of the Society for Neuroscience
|January 8, 2010
PubMed
Summary

CollagenQ (ColQ) anchors acetylcholinesterase at neuromuscular junctions. Its absence alters acetylcholine receptor clustering and signaling via MuSK, revealing a regulatory role beyond structure.

More Related Videos

Characterization of Neuromuscular Junctions in Mice by Combined Confocal and Super-Resolution Microscopy
11:03

Characterization of Neuromuscular Junctions in Mice by Combined Confocal and Super-Resolution Microscopy

Published on: December 8, 2021

Historical View and Physiology Demonstration at the NMJ of the Crayfish Opener Muscle
11:56

Historical View and Physiology Demonstration at the NMJ of the Crayfish Opener Muscle

Published on: November 9, 2009

Related Experiment Videos

Last Updated: Jun 17, 2026

Physiological Recordings of High and Low Output NMJs on the Crayfish Leg Extensor Muscle
10:00

Physiological Recordings of High and Low Output NMJs on the Crayfish Leg Extensor Muscle

Published on: November 17, 2010

Characterization of Neuromuscular Junctions in Mice by Combined Confocal and Super-Resolution Microscopy
11:03

Characterization of Neuromuscular Junctions in Mice by Combined Confocal and Super-Resolution Microscopy

Published on: December 8, 2021

Historical View and Physiology Demonstration at the NMJ of the Crayfish Opener Muscle
11:56

Historical View and Physiology Demonstration at the NMJ of the Crayfish Opener Muscle

Published on: November 9, 2009

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Cell Biology

Background:

  • CollagenQ (ColQ) is crucial for neuromuscular junction (NMJ) structure, anchoring acetylcholinesterase (AChE) and interacting with MuSK.
  • MuSK signaling is vital for acetylcholine receptor (AChR) clustering and synaptic gene regulation.

Purpose of the Study:

  • To investigate the regulatory role of ColQ in synaptic protein clustering and expression.
  • To compare synaptic protein dynamics in wild-type versus ColQ-deficient muscle cells in vitro and in vivo.

Main Methods:

  • Comparative analysis of muscle cells in culture and at the NMJ.
  • Assessment of AChR clustering, MuSK mRNA and membrane-bound levels.
  • Investigation of MuSK signaling pathway activation and AChR subunit phosphorylation.

Main Results:

  • AChR clusters were smaller and denser in ColQ-deficient cells.
  • Membrane-bound MuSK decreased, while MuSK mRNA increased in cultured cells lacking ColQ.
  • ColQ deficiency altered MuSK signaling, affecting AChR clustering, beta-AChR phosphorylation, and AChR mRNA levels.

Conclusions:

  • ColQ has a regulatory role at the synapse, influencing AChR clustering and synaptic gene expression.
  • ColQ interacts with MuSK to control MuSK localization and signaling pathway activation.
  • These findings highlight ColQ's dual structural and regulatory functions at the NMJ.